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Hani N Sabbah - One of the best experts on this subject based on the ideXlab platform.

  • effects of Elamipretide on left ventricular function in patients with heart failure with reduced ejection fraction the progress hf phase 2 trial
    Journal of Cardiac Failure, 2020
    Co-Authors: Javed Butler, Christopher M Oconnor, Hani N Sabbah, Muhammad Shahzeb Khan, Stefan D Anker, Gregg C Fonarow, Savina Nodari, Burkert Pieske, Elisabeth Pieskekraigher, Michele Senni
    Abstract:

    Abstract Background Elamipretide, a novel mitochondrial modulating agent, improves myocardial energetics, however, it is unknown whether this mechanistic benefit translates into improved cardiac structure and function in HF with reduced ejection fraction (HFrEF). The objective of this study was to evaluate the effects of multiple subcutaneous doses of Elamipretide on left ventricular end systolic volume (LVESV) assessed by cardiac magnetic resonance imaging (MRI). Methods Seventy-one HFrEF (LVEF ≤ 40%) patients were randomized in a double-blind, placebo-controlled trial in a 1:1:1 ratio to receive either placebo, 4 mg or 40 mg Elamipretide once daily for 28 consecutive days. Results Mean age (SD) of the study population was 65±10 years, 24% were females, and mean EF was 31±7%. The change in LVESV from baseline to week 4 was not significantly different between Elamipretide 4mg (89.4 ml to 85 ml; difference, −4.4 ml) or 40 mg (77.9 ml to 76.6 ml; difference, −1.2 ml) compared with placebo (77.7 ml to 74.6 ml; difference, −3.8 ml) [4mg versus placebo: difference of means, −0.3; 95% CI, −4.6 to 4.0; P = 0.90; and 40mg versus placebo: difference of means, 2.3; 95% CI, −1.9 to 6.5; P = 0.28]. Also, no significant differences in change in left ventricular end diastolic volume and left ventricular ejection fraction were observed between placebo and either of the Elamipretide group. Rates of any study drug related adverse events were similar among the three groups. Conclusions Elamipretide was well tolerated but did not improve LVESV at 4 weeks in stable HFrEF patients compared with placebo.

  • Elamipretide reduces the burden for induction of apoptosis in skeletal muscle of dogs with chronic heart failure
    Journal of the American College of Cardiology, 2019
    Co-Authors: Hani N Sabbah, Ramesh C Gupta, Kefei Zhang, Jiang Xu, Vinita Singhgupta
    Abstract:

    Exercise intolerance (Ex-Int) is a feature of heart failure (HF) and is attributed to skeletal muscle (SM) abnormalities including fiber type composition, atrophy, fiber loss, and mitochondrial (MITO) dysfunction. MITO dysfunction can activate apoptosis by releasing cytochrome C (CYTO-C) into the

  • effects of Elamipretide on skeletal muscle in dogs with experimentally induced heart failure
    Esc Heart Failure, 2019
    Co-Authors: Hani N Sabbah, Ramesh C Gupta, Vinita Singhgupta, Kefei Zhang
    Abstract:

    AIMS: Elamipretide (ELAM), an aromatic-cationic tetrapeptide, interacts with cardiolipin and normalizes dysfunctional mitochondria of cardiomyocytes. This study examined the effects of ELAM on skeletal muscle mitochondria function in dogs with chronic heart failure (HF). METHODS AND RESULTS: Studies were performed in skeletal muscle biopsy specimens obtained from normal dogs (n = 7) and dogs with chronic intracoronary microembolization-induced HF (n = 14) treated with subcutaneous ELAM 0.5 mg/kg (HF + ELAM, n = 7) or vehicle (normal saline control, HF-CON, n = 7). After 3 months of therapy, triceps skeletal muscle samples were obtained from all dogs, and the proportion of type 1 and type 2 fibres was assessed. Mitochondria isolated from myofibrils of the vastus lateralis skeletal muscle exposed in vitro to ELAM for 1 h were used to assess mitochondrial function. The proportion of skeletal muscle type 1 fibres was lower in HF-CON dogs compared with normal dogs (23 ± 4 vs. 32 ± 5%, P < 0.05). Treatment with ELAM restored a near-normal fibre-type composition (31 ± 7%, P < 0.05 vs. HF-CON). Skeletal muscle mitochondria showed significantly lower levels of adenosine diphosphate-dependent mitochondrial respiration (100 ± 9 vs. 164 ± 15 natom O/min/mg protein, P < 0.05), mitochondrial membrane potential (0.17 ± 0.03 vs. 0.53 ± 0.03 red/green fluorescence ratio, P < 0.05), mitochondrial permeability transition pore (38 ± 3 vs. 62 ± 2 relative light units, P < 0.05), maximum rate of adenosine triphosphate synthesis (3284 ± 418 vs. 8835 ± 423 RLU/μg protein, P < 0.05), and cytochrome c oxidase activity (1390 ± 108 vs. 2459 ± 210 natom O/min/mg protein, P < 0.05) compared with normal dogs. Exposure of skeletal muscle myofibrillar mitochondria from HF dogs to ELAM showed a dose-dependent improvement/normalization of all measures of mitochondrial function. In mitochondria from skeletal muscle of HF dogs exposed to 0.10 μM ELAM, adenosine diphosphate-dependent mitochondrial respiration increased to 183 ± 18 natom O/min/mg protein, membrane potential increased to 0.30 ± 0.03 red/green fluorescence ratio, mitochondrial permeability transition pore increased to 54 ± 4 RLU, maximum rate of adenosine triphosphate synthesis increased to 4423 ± 414, and cytochrome c oxidase activity increased to 2033 ± 191 natom O/min/mg protein. Exposure of skeletal muscle myofibrillar mitochondria from normal dogs to ELAM had no effect on mitochondrial function parameters. CONCLUSIONS: The results indicate that ELAM, previously shown to positively influence mitochondrial function of the failing heart, can also positively impact mitochondrial function of skeletal muscle and potentially help restore skeletal muscle function and improve exercise tolerance.

  • Effects of Elamipretide on skeletal muscle in dogs with experimentally induced heart failure.
    Esc Heart Failure, 2019
    Co-Authors: Hani N Sabbah, Ramesh C Gupta, Vinita Singh-gupta, Kefei Zhang
    Abstract:

    Aims Elamipretide (ELAM), an aromatic–cationic tetrapeptide, interacts with cardiolipin and normalizes dysfunctional mitochondria of cardiomyocytes. This study examined the effects of ELAM on skeletal muscle mitochondria function in dogs with chronic heart failure (HF).

  • Improving Mitochondrial Function Improves the Plasma Metabolite Profile in Experimental Heart Failure
    Journal of Cardiac Failure, 2018
    Co-Authors: Jia Li, Hani N Sabbah, L. Keoki Williams, Stephen J. Gardell, David E. Lanfear
    Abstract:

    Background We recently reported a strong association of plasma metabolite profile with survival in patients with heart failure and reduced ejection fraction (HFrEF). Mitochondrial function is perturbed in HFrEF, but whether plasma metabolite levels are linked to mitochondrial dysfunction is unknown. Elamipretide, a 4 amino acid peptide, normalizes mitochondrial energy production and thus provides a tool to test the association of mitochondrial function with the plasma metabolome in HFrEF. Methods Dogs (n=14) underwent intracoronary micro-embolization to induce HFrEF (pre-treatment EF 35%) and then were randomized 1:1 to Elamipretide or placebo for 3 months. Targeted plasma metabolomic profiling was performed to quantitate 8 organic acids (OA), 23 amino acids (AA), and up to 59 acylcarnitines (AC) at pre-treatment and end of study. The association of changes in metabolite levels with treatment were tested using the Wilcoxon rank sum test on the principal components (PC) of each of the three metabolite classes. We also tested the correlation of metabolite levels with mitochondrial maximal rate of ATP synthesis in HF dog myocardium. We then attempted to confirm associations of interest in a human HFrEF cohort (n=1060) using bivariate Cox models. Result There was no association of plasma AA or AC changes with Elamipretide treatment. However, there was a near-significant association of the first PC of OA changes (p=0.073), with treatment leading to lower plasma levels of OA compared to placebo (Figure). The OA also trended towards inverse relationship to maximal rate of ATP synthesis in dogs (correlation coef -0.09 to -0.67, mean -0.29), pyruvate having the strongest association (Coef =-0.67, p=0.012). In humans with HFrEF, directionally congruent and statistically significant associations with mortality risk were seen for 6 of these 8 OA (Table). Conclusion In dogs with HFrEF improving mitochondrial function using Elamipretide resulted in favorable changes in plasma metabolites, specifically OA, which were risk-associated in human HFrEF. Our data suggest that mitochondrial dysfunction is the mechanism linking these plasma metabolomic abnormalities with worse survival in HFrEF.

Kefei Zhang - One of the best experts on this subject based on the ideXlab platform.

  • Elamipretide reduces the burden for induction of apoptosis in skeletal muscle of dogs with chronic heart failure
    Journal of the American College of Cardiology, 2019
    Co-Authors: Hani N Sabbah, Ramesh C Gupta, Kefei Zhang, Jiang Xu, Vinita Singhgupta
    Abstract:

    Exercise intolerance (Ex-Int) is a feature of heart failure (HF) and is attributed to skeletal muscle (SM) abnormalities including fiber type composition, atrophy, fiber loss, and mitochondrial (MITO) dysfunction. MITO dysfunction can activate apoptosis by releasing cytochrome C (CYTO-C) into the

  • effects of Elamipretide on skeletal muscle in dogs with experimentally induced heart failure
    Esc Heart Failure, 2019
    Co-Authors: Hani N Sabbah, Ramesh C Gupta, Vinita Singhgupta, Kefei Zhang
    Abstract:

    AIMS: Elamipretide (ELAM), an aromatic-cationic tetrapeptide, interacts with cardiolipin and normalizes dysfunctional mitochondria of cardiomyocytes. This study examined the effects of ELAM on skeletal muscle mitochondria function in dogs with chronic heart failure (HF). METHODS AND RESULTS: Studies were performed in skeletal muscle biopsy specimens obtained from normal dogs (n = 7) and dogs with chronic intracoronary microembolization-induced HF (n = 14) treated with subcutaneous ELAM 0.5 mg/kg (HF + ELAM, n = 7) or vehicle (normal saline control, HF-CON, n = 7). After 3 months of therapy, triceps skeletal muscle samples were obtained from all dogs, and the proportion of type 1 and type 2 fibres was assessed. Mitochondria isolated from myofibrils of the vastus lateralis skeletal muscle exposed in vitro to ELAM for 1 h were used to assess mitochondrial function. The proportion of skeletal muscle type 1 fibres was lower in HF-CON dogs compared with normal dogs (23 ± 4 vs. 32 ± 5%, P < 0.05). Treatment with ELAM restored a near-normal fibre-type composition (31 ± 7%, P < 0.05 vs. HF-CON). Skeletal muscle mitochondria showed significantly lower levels of adenosine diphosphate-dependent mitochondrial respiration (100 ± 9 vs. 164 ± 15 natom O/min/mg protein, P < 0.05), mitochondrial membrane potential (0.17 ± 0.03 vs. 0.53 ± 0.03 red/green fluorescence ratio, P < 0.05), mitochondrial permeability transition pore (38 ± 3 vs. 62 ± 2 relative light units, P < 0.05), maximum rate of adenosine triphosphate synthesis (3284 ± 418 vs. 8835 ± 423 RLU/μg protein, P < 0.05), and cytochrome c oxidase activity (1390 ± 108 vs. 2459 ± 210 natom O/min/mg protein, P < 0.05) compared with normal dogs. Exposure of skeletal muscle myofibrillar mitochondria from HF dogs to ELAM showed a dose-dependent improvement/normalization of all measures of mitochondrial function. In mitochondria from skeletal muscle of HF dogs exposed to 0.10 μM ELAM, adenosine diphosphate-dependent mitochondrial respiration increased to 183 ± 18 natom O/min/mg protein, membrane potential increased to 0.30 ± 0.03 red/green fluorescence ratio, mitochondrial permeability transition pore increased to 54 ± 4 RLU, maximum rate of adenosine triphosphate synthesis increased to 4423 ± 414, and cytochrome c oxidase activity increased to 2033 ± 191 natom O/min/mg protein. Exposure of skeletal muscle myofibrillar mitochondria from normal dogs to ELAM had no effect on mitochondrial function parameters. CONCLUSIONS: The results indicate that ELAM, previously shown to positively influence mitochondrial function of the failing heart, can also positively impact mitochondrial function of skeletal muscle and potentially help restore skeletal muscle function and improve exercise tolerance.

  • Effects of Elamipretide on skeletal muscle in dogs with experimentally induced heart failure.
    Esc Heart Failure, 2019
    Co-Authors: Hani N Sabbah, Ramesh C Gupta, Vinita Singh-gupta, Kefei Zhang
    Abstract:

    Aims Elamipretide (ELAM), an aromatic–cationic tetrapeptide, interacts with cardiolipin and normalizes dysfunctional mitochondria of cardiomyocytes. This study examined the effects of ELAM on skeletal muscle mitochondria function in dogs with chronic heart failure (HF).

  • Abnormalities of Mitochondrial Dynamics in the Failing Heart: Normalization Following Long-Term Therapy with Elamipretide
    Cardiovascular Drugs and Therapy, 2018
    Co-Authors: Hani N Sabbah, Ramesh C Gupta, Kefei Zhang, Vinita Singh-gupta, David E. Lanfear
    Abstract:

    Purpose Abnormalities of MITO dynamics occur in HF and have been implicated in disease progression. This study describes the broad range abnormalities of mitochondrial (MITO) dynamics in Heart Failure with reduced ejection fraction (HF) and evaluates the effects of long-term therapy with Elamipretide (ELAM), a MITO-targeting peptide, on these abnormalities. Methods Studies were performed in left ventricular tissue from dogs and humans with HF, and were compared with tissue from healthy dogs and healthy donor human hearts. Dogs with HF were randomized to 3 months therapy with ELAM or vehicle. The following were evaluated in dog and human hearts: (1) regulators of MITO biogenesis, including endothelial nitric oxide synthase (eNOS), cyclic guanosine monophosphate (cGMP), and peroxisome proliferator-activated receptor gamma coactivator 1α (PGC-1α, a transcription factor that drives MITO biogenesis); (2) regulators of MITO fission and fusion, including fission-1, dynamin-related protein-1, mitofusion-2, dominant optic atrophy-1, and mitofilin; and (3) determinants of cardiolipin (CL) synthesis and remodeling, including CL synthase-1, tafazzin-1, and acyl-CoA:lysocardiolipin acyltransferase-1. Results The study showed decreased levels of eNOS, cGMP, and PGC-1α in HF (dog and human). Increased levels of fission-associated proteins, decreased levels of fusion-associated proteins, decreased mitofilin, and abnormalities of CL synthesis and remodeling were also observed. In all instances, these maladaptations were normalized following long-term therapy with ELAM. Conclusions Critical abnormalities of MITO dynamics occur in HF and are normalized following long-term therapy with ELAM. The findings provide support for the continued development of ELAM for the treatment of HF.

  • abnormalities of mitochondrial dynamics in the failing heart normalization following long term therapy with Elamipretide
    Cardiovascular Drugs and Therapy, 2018
    Co-Authors: Hani N Sabbah, Ramesh C Gupta, Kefei Zhang, Vinita Singhgupta, David E. Lanfear
    Abstract:

    Abnormalities of MITO dynamics occur in HF and have been implicated in disease progression. This study describes the broad range abnormalities of mitochondrial (MITO) dynamics in Heart Failure with reduced ejection fraction (HF) and evaluates the effects of long-term therapy with Elamipretide (ELAM), a MITO-targeting peptide, on these abnormalities. Studies were performed in left ventricular tissue from dogs and humans with HF, and were compared with tissue from healthy dogs and healthy donor human hearts. Dogs with HF were randomized to 3 months therapy with ELAM or vehicle. The following were evaluated in dog and human hearts: (1) regulators of MITO biogenesis, including endothelial nitric oxide synthase (eNOS), cyclic guanosine monophosphate (cGMP), and peroxisome proliferator-activated receptor gamma coactivator 1α (PGC-1α, a transcription factor that drives MITO biogenesis); (2) regulators of MITO fission and fusion, including fission-1, dynamin-related protein-1, mitofusion-2, dominant optic atrophy-1, and mitofilin; and (3) determinants of cardiolipin (CL) synthesis and remodeling, including CL synthase-1, tafazzin-1, and acyl-CoA:lysocardiolipin acyltransferase-1. The study showed decreased levels of eNOS, cGMP, and PGC-1α in HF (dog and human). Increased levels of fission-associated proteins, decreased levels of fusion-associated proteins, decreased mitofilin, and abnormalities of CL synthesis and remodeling were also observed. In all instances, these maladaptations were normalized following long-term therapy with ELAM. Critical abnormalities of MITO dynamics occur in HF and are normalized following long-term therapy with ELAM. The findings provide support for the continued development of ELAM for the treatment of HF.

Ramesh C Gupta - One of the best experts on this subject based on the ideXlab platform.

  • Elamipretide reduces the burden for induction of apoptosis in skeletal muscle of dogs with chronic heart failure
    Journal of the American College of Cardiology, 2019
    Co-Authors: Hani N Sabbah, Ramesh C Gupta, Kefei Zhang, Jiang Xu, Vinita Singhgupta
    Abstract:

    Exercise intolerance (Ex-Int) is a feature of heart failure (HF) and is attributed to skeletal muscle (SM) abnormalities including fiber type composition, atrophy, fiber loss, and mitochondrial (MITO) dysfunction. MITO dysfunction can activate apoptosis by releasing cytochrome C (CYTO-C) into the

  • effects of Elamipretide on skeletal muscle in dogs with experimentally induced heart failure
    Esc Heart Failure, 2019
    Co-Authors: Hani N Sabbah, Ramesh C Gupta, Vinita Singhgupta, Kefei Zhang
    Abstract:

    AIMS: Elamipretide (ELAM), an aromatic-cationic tetrapeptide, interacts with cardiolipin and normalizes dysfunctional mitochondria of cardiomyocytes. This study examined the effects of ELAM on skeletal muscle mitochondria function in dogs with chronic heart failure (HF). METHODS AND RESULTS: Studies were performed in skeletal muscle biopsy specimens obtained from normal dogs (n = 7) and dogs with chronic intracoronary microembolization-induced HF (n = 14) treated with subcutaneous ELAM 0.5 mg/kg (HF + ELAM, n = 7) or vehicle (normal saline control, HF-CON, n = 7). After 3 months of therapy, triceps skeletal muscle samples were obtained from all dogs, and the proportion of type 1 and type 2 fibres was assessed. Mitochondria isolated from myofibrils of the vastus lateralis skeletal muscle exposed in vitro to ELAM for 1 h were used to assess mitochondrial function. The proportion of skeletal muscle type 1 fibres was lower in HF-CON dogs compared with normal dogs (23 ± 4 vs. 32 ± 5%, P < 0.05). Treatment with ELAM restored a near-normal fibre-type composition (31 ± 7%, P < 0.05 vs. HF-CON). Skeletal muscle mitochondria showed significantly lower levels of adenosine diphosphate-dependent mitochondrial respiration (100 ± 9 vs. 164 ± 15 natom O/min/mg protein, P < 0.05), mitochondrial membrane potential (0.17 ± 0.03 vs. 0.53 ± 0.03 red/green fluorescence ratio, P < 0.05), mitochondrial permeability transition pore (38 ± 3 vs. 62 ± 2 relative light units, P < 0.05), maximum rate of adenosine triphosphate synthesis (3284 ± 418 vs. 8835 ± 423 RLU/μg protein, P < 0.05), and cytochrome c oxidase activity (1390 ± 108 vs. 2459 ± 210 natom O/min/mg protein, P < 0.05) compared with normal dogs. Exposure of skeletal muscle myofibrillar mitochondria from HF dogs to ELAM showed a dose-dependent improvement/normalization of all measures of mitochondrial function. In mitochondria from skeletal muscle of HF dogs exposed to 0.10 μM ELAM, adenosine diphosphate-dependent mitochondrial respiration increased to 183 ± 18 natom O/min/mg protein, membrane potential increased to 0.30 ± 0.03 red/green fluorescence ratio, mitochondrial permeability transition pore increased to 54 ± 4 RLU, maximum rate of adenosine triphosphate synthesis increased to 4423 ± 414, and cytochrome c oxidase activity increased to 2033 ± 191 natom O/min/mg protein. Exposure of skeletal muscle myofibrillar mitochondria from normal dogs to ELAM had no effect on mitochondrial function parameters. CONCLUSIONS: The results indicate that ELAM, previously shown to positively influence mitochondrial function of the failing heart, can also positively impact mitochondrial function of skeletal muscle and potentially help restore skeletal muscle function and improve exercise tolerance.

  • Effects of Elamipretide on skeletal muscle in dogs with experimentally induced heart failure.
    Esc Heart Failure, 2019
    Co-Authors: Hani N Sabbah, Ramesh C Gupta, Vinita Singh-gupta, Kefei Zhang
    Abstract:

    Aims Elamipretide (ELAM), an aromatic–cationic tetrapeptide, interacts with cardiolipin and normalizes dysfunctional mitochondria of cardiomyocytes. This study examined the effects of ELAM on skeletal muscle mitochondria function in dogs with chronic heart failure (HF).

  • Abnormalities of Mitochondrial Dynamics in the Failing Heart: Normalization Following Long-Term Therapy with Elamipretide
    Cardiovascular Drugs and Therapy, 2018
    Co-Authors: Hani N Sabbah, Ramesh C Gupta, Kefei Zhang, Vinita Singh-gupta, David E. Lanfear
    Abstract:

    Purpose Abnormalities of MITO dynamics occur in HF and have been implicated in disease progression. This study describes the broad range abnormalities of mitochondrial (MITO) dynamics in Heart Failure with reduced ejection fraction (HF) and evaluates the effects of long-term therapy with Elamipretide (ELAM), a MITO-targeting peptide, on these abnormalities. Methods Studies were performed in left ventricular tissue from dogs and humans with HF, and were compared with tissue from healthy dogs and healthy donor human hearts. Dogs with HF were randomized to 3 months therapy with ELAM or vehicle. The following were evaluated in dog and human hearts: (1) regulators of MITO biogenesis, including endothelial nitric oxide synthase (eNOS), cyclic guanosine monophosphate (cGMP), and peroxisome proliferator-activated receptor gamma coactivator 1α (PGC-1α, a transcription factor that drives MITO biogenesis); (2) regulators of MITO fission and fusion, including fission-1, dynamin-related protein-1, mitofusion-2, dominant optic atrophy-1, and mitofilin; and (3) determinants of cardiolipin (CL) synthesis and remodeling, including CL synthase-1, tafazzin-1, and acyl-CoA:lysocardiolipin acyltransferase-1. Results The study showed decreased levels of eNOS, cGMP, and PGC-1α in HF (dog and human). Increased levels of fission-associated proteins, decreased levels of fusion-associated proteins, decreased mitofilin, and abnormalities of CL synthesis and remodeling were also observed. In all instances, these maladaptations were normalized following long-term therapy with ELAM. Conclusions Critical abnormalities of MITO dynamics occur in HF and are normalized following long-term therapy with ELAM. The findings provide support for the continued development of ELAM for the treatment of HF.

  • abnormalities of mitochondrial dynamics in the failing heart normalization following long term therapy with Elamipretide
    Cardiovascular Drugs and Therapy, 2018
    Co-Authors: Hani N Sabbah, Ramesh C Gupta, Kefei Zhang, Vinita Singhgupta, David E. Lanfear
    Abstract:

    Abnormalities of MITO dynamics occur in HF and have been implicated in disease progression. This study describes the broad range abnormalities of mitochondrial (MITO) dynamics in Heart Failure with reduced ejection fraction (HF) and evaluates the effects of long-term therapy with Elamipretide (ELAM), a MITO-targeting peptide, on these abnormalities. Studies were performed in left ventricular tissue from dogs and humans with HF, and were compared with tissue from healthy dogs and healthy donor human hearts. Dogs with HF were randomized to 3 months therapy with ELAM or vehicle. The following were evaluated in dog and human hearts: (1) regulators of MITO biogenesis, including endothelial nitric oxide synthase (eNOS), cyclic guanosine monophosphate (cGMP), and peroxisome proliferator-activated receptor gamma coactivator 1α (PGC-1α, a transcription factor that drives MITO biogenesis); (2) regulators of MITO fission and fusion, including fission-1, dynamin-related protein-1, mitofusion-2, dominant optic atrophy-1, and mitofilin; and (3) determinants of cardiolipin (CL) synthesis and remodeling, including CL synthase-1, tafazzin-1, and acyl-CoA:lysocardiolipin acyltransferase-1. The study showed decreased levels of eNOS, cGMP, and PGC-1α in HF (dog and human). Increased levels of fission-associated proteins, decreased levels of fusion-associated proteins, decreased mitofilin, and abnormalities of CL synthesis and remodeling were also observed. In all instances, these maladaptations were normalized following long-term therapy with ELAM. Critical abnormalities of MITO dynamics occur in HF and are normalized following long-term therapy with ELAM. The findings provide support for the continued development of ELAM for the treatment of HF.

Vinita Singhgupta - One of the best experts on this subject based on the ideXlab platform.

  • Elamipretide reduces the burden for induction of apoptosis in skeletal muscle of dogs with chronic heart failure
    Journal of the American College of Cardiology, 2019
    Co-Authors: Hani N Sabbah, Ramesh C Gupta, Kefei Zhang, Jiang Xu, Vinita Singhgupta
    Abstract:

    Exercise intolerance (Ex-Int) is a feature of heart failure (HF) and is attributed to skeletal muscle (SM) abnormalities including fiber type composition, atrophy, fiber loss, and mitochondrial (MITO) dysfunction. MITO dysfunction can activate apoptosis by releasing cytochrome C (CYTO-C) into the

  • effects of Elamipretide on skeletal muscle in dogs with experimentally induced heart failure
    Esc Heart Failure, 2019
    Co-Authors: Hani N Sabbah, Ramesh C Gupta, Vinita Singhgupta, Kefei Zhang
    Abstract:

    AIMS: Elamipretide (ELAM), an aromatic-cationic tetrapeptide, interacts with cardiolipin and normalizes dysfunctional mitochondria of cardiomyocytes. This study examined the effects of ELAM on skeletal muscle mitochondria function in dogs with chronic heart failure (HF). METHODS AND RESULTS: Studies were performed in skeletal muscle biopsy specimens obtained from normal dogs (n = 7) and dogs with chronic intracoronary microembolization-induced HF (n = 14) treated with subcutaneous ELAM 0.5 mg/kg (HF + ELAM, n = 7) or vehicle (normal saline control, HF-CON, n = 7). After 3 months of therapy, triceps skeletal muscle samples were obtained from all dogs, and the proportion of type 1 and type 2 fibres was assessed. Mitochondria isolated from myofibrils of the vastus lateralis skeletal muscle exposed in vitro to ELAM for 1 h were used to assess mitochondrial function. The proportion of skeletal muscle type 1 fibres was lower in HF-CON dogs compared with normal dogs (23 ± 4 vs. 32 ± 5%, P < 0.05). Treatment with ELAM restored a near-normal fibre-type composition (31 ± 7%, P < 0.05 vs. HF-CON). Skeletal muscle mitochondria showed significantly lower levels of adenosine diphosphate-dependent mitochondrial respiration (100 ± 9 vs. 164 ± 15 natom O/min/mg protein, P < 0.05), mitochondrial membrane potential (0.17 ± 0.03 vs. 0.53 ± 0.03 red/green fluorescence ratio, P < 0.05), mitochondrial permeability transition pore (38 ± 3 vs. 62 ± 2 relative light units, P < 0.05), maximum rate of adenosine triphosphate synthesis (3284 ± 418 vs. 8835 ± 423 RLU/μg protein, P < 0.05), and cytochrome c oxidase activity (1390 ± 108 vs. 2459 ± 210 natom O/min/mg protein, P < 0.05) compared with normal dogs. Exposure of skeletal muscle myofibrillar mitochondria from HF dogs to ELAM showed a dose-dependent improvement/normalization of all measures of mitochondrial function. In mitochondria from skeletal muscle of HF dogs exposed to 0.10 μM ELAM, adenosine diphosphate-dependent mitochondrial respiration increased to 183 ± 18 natom O/min/mg protein, membrane potential increased to 0.30 ± 0.03 red/green fluorescence ratio, mitochondrial permeability transition pore increased to 54 ± 4 RLU, maximum rate of adenosine triphosphate synthesis increased to 4423 ± 414, and cytochrome c oxidase activity increased to 2033 ± 191 natom O/min/mg protein. Exposure of skeletal muscle myofibrillar mitochondria from normal dogs to ELAM had no effect on mitochondrial function parameters. CONCLUSIONS: The results indicate that ELAM, previously shown to positively influence mitochondrial function of the failing heart, can also positively impact mitochondrial function of skeletal muscle and potentially help restore skeletal muscle function and improve exercise tolerance.

  • abnormalities of mitochondrial dynamics in the failing heart normalization following long term therapy with Elamipretide
    Cardiovascular Drugs and Therapy, 2018
    Co-Authors: Hani N Sabbah, Ramesh C Gupta, Kefei Zhang, Vinita Singhgupta, David E. Lanfear
    Abstract:

    Abnormalities of MITO dynamics occur in HF and have been implicated in disease progression. This study describes the broad range abnormalities of mitochondrial (MITO) dynamics in Heart Failure with reduced ejection fraction (HF) and evaluates the effects of long-term therapy with Elamipretide (ELAM), a MITO-targeting peptide, on these abnormalities. Studies were performed in left ventricular tissue from dogs and humans with HF, and were compared with tissue from healthy dogs and healthy donor human hearts. Dogs with HF were randomized to 3 months therapy with ELAM or vehicle. The following were evaluated in dog and human hearts: (1) regulators of MITO biogenesis, including endothelial nitric oxide synthase (eNOS), cyclic guanosine monophosphate (cGMP), and peroxisome proliferator-activated receptor gamma coactivator 1α (PGC-1α, a transcription factor that drives MITO biogenesis); (2) regulators of MITO fission and fusion, including fission-1, dynamin-related protein-1, mitofusion-2, dominant optic atrophy-1, and mitofilin; and (3) determinants of cardiolipin (CL) synthesis and remodeling, including CL synthase-1, tafazzin-1, and acyl-CoA:lysocardiolipin acyltransferase-1. The study showed decreased levels of eNOS, cGMP, and PGC-1α in HF (dog and human). Increased levels of fission-associated proteins, decreased levels of fusion-associated proteins, decreased mitofilin, and abnormalities of CL synthesis and remodeling were also observed. In all instances, these maladaptations were normalized following long-term therapy with ELAM. Critical abnormalities of MITO dynamics occur in HF and are normalized following long-term therapy with ELAM. The findings provide support for the continued development of ELAM for the treatment of HF.

  • Elamipretide restores protein and mrna expression levels of cardiolipin synthase 1 in left ventricular myocardium of dogs with chronic heart failure
    Journal of the American College of Cardiology, 2018
    Co-Authors: Ramesh C Gupta, Kefei Zhang, Vinita Singhgupta, Jiang Xu, Hani N Sabbah
    Abstract:

    Cardiolipin (CL), an inner mitochondrial (MITO) membrane phospholipid is synthesized by CL synthase-1 (CLS1) and is critical for normal MITO function. Levels of CL are reduced in MITO of LV myocardium of dogs with heart failure (HF) and are normalized after chronic therapy with Elamipretide (ELAM),

  • long term therapy with Elamipretide normalizes sirtuin 3 protein levels in isolated mitochondria of left ventricular myocardium of dogs with chronic heart failure
    Journal of the American College of Cardiology, 2018
    Co-Authors: Ramesh C Gupta, Vinita Singhgupta, Kristina Castle, Hani N Sabbah
    Abstract:

    Sirtuins are a highly conserved family of histone/protein deacetylases. In mammalian cells, seven sirtuins (SIRT1-7) modulate distinct metabolic and stress-response pathways, with SIRT3 having been most extensively investigated in the cardiovascular system and is mainly located in the mitochondria.

Jiang Xu - One of the best experts on this subject based on the ideXlab platform.