The Experts below are selected from a list of 9 Experts worldwide ranked by ideXlab platform

William H. Griffith - One of the best experts on this subject based on the ideXlab platform.

  • Homeostatic compensation maintains Ca2+ signaling functions in Purkinje neurons in the Leaner Mutant Mouse.
    Cerebellum (London England), 2002
    Co-Authors: David Murchison, Leonard S. Dove, Louise C. Abbott, William H. Griffith
    Abstract:

    Several human neurological disorders have been associated with mutations in the gene coding for the 1 subunit of the P/Q type voltage-gated calcium channel (α1A/CaV2.1). Mutations in this gene also occur in a number of neurolog-ically afflected Mouse strains, including Leaner (tgla/tgla). Because the P-type calcium current is very prominent in cerebellar Purkinje neurons, these cells from mice with α1 subunit mutations make excellent models for the investigation of the functional consequences of native mutations in a voltage-gated calcium channel of mammalian central nervous system. In this review, we describe the impact of altered channel function on cellular calcium homeostasis and signaling. Remarkably, calcium buffering functions of the endoplasmic reticulum and calcium-binding proteins appear to be regulated in order to compensate for altered calcium influx through the Mutant channels. Although this compensation may serve to maintain calcium signaling functions, such as calcium-induced calcium release, it remains uncertain whether such compensation alleviates or contributes to the behavioral phenotype.

  • Homeostatic compensation maintains Ca^2+ signaling functions in Purkinje neurons in the Leaner Mutant Mouse
    The Cerebellum, 2002
    Co-Authors: David Murchison, Leonard S. Dove, Louise C. Abbott, William H. Griffith
    Abstract:

    Several human neurological disorders have been associated with mutations in the gene coding for the 1 subunit of the P/Q type voltage-gated calcium channel (α1_A/Ca_V2.1). Mutations in this gene also occur in a number of neurolog-ically afflected Mouse strains, including Leaner (tg^la/tg^la). Because the P-type calcium current is very prominent in cerebellar Purkinje neurons, these cells from mice with α1 subunit mutations make excellent models for the investigation of the functional consequences of native mutations in a voltage-gated calcium channel of mammalian central nervous system. In this review, we describe the impact of altered channel function on cellular calcium homeostasis and signaling. Remarkably, calcium buffering functions of the endoplasmic reticulum and calcium-binding proteins appear to be regulated in order to compensate for altered calcium influx through the Mutant channels. Although this compensation may serve to maintain calcium signaling functions, such as calcium-induced calcium release, it remains uncertain whether such compensation alleviates or contributes to the behavioral phenotype.

David Murchison - One of the best experts on this subject based on the ideXlab platform.

  • Homeostatic compensation maintains Ca2+ signaling functions in Purkinje neurons in the Leaner Mutant Mouse.
    Cerebellum (London England), 2002
    Co-Authors: David Murchison, Leonard S. Dove, Louise C. Abbott, William H. Griffith
    Abstract:

    Several human neurological disorders have been associated with mutations in the gene coding for the 1 subunit of the P/Q type voltage-gated calcium channel (α1A/CaV2.1). Mutations in this gene also occur in a number of neurolog-ically afflected Mouse strains, including Leaner (tgla/tgla). Because the P-type calcium current is very prominent in cerebellar Purkinje neurons, these cells from mice with α1 subunit mutations make excellent models for the investigation of the functional consequences of native mutations in a voltage-gated calcium channel of mammalian central nervous system. In this review, we describe the impact of altered channel function on cellular calcium homeostasis and signaling. Remarkably, calcium buffering functions of the endoplasmic reticulum and calcium-binding proteins appear to be regulated in order to compensate for altered calcium influx through the Mutant channels. Although this compensation may serve to maintain calcium signaling functions, such as calcium-induced calcium release, it remains uncertain whether such compensation alleviates or contributes to the behavioral phenotype.

  • Homeostatic compensation maintains Ca^2+ signaling functions in Purkinje neurons in the Leaner Mutant Mouse
    The Cerebellum, 2002
    Co-Authors: David Murchison, Leonard S. Dove, Louise C. Abbott, William H. Griffith
    Abstract:

    Several human neurological disorders have been associated with mutations in the gene coding for the 1 subunit of the P/Q type voltage-gated calcium channel (α1_A/Ca_V2.1). Mutations in this gene also occur in a number of neurolog-ically afflected Mouse strains, including Leaner (tg^la/tg^la). Because the P-type calcium current is very prominent in cerebellar Purkinje neurons, these cells from mice with α1 subunit mutations make excellent models for the investigation of the functional consequences of native mutations in a voltage-gated calcium channel of mammalian central nervous system. In this review, we describe the impact of altered channel function on cellular calcium homeostasis and signaling. Remarkably, calcium buffering functions of the endoplasmic reticulum and calcium-binding proteins appear to be regulated in order to compensate for altered calcium influx through the Mutant channels. Although this compensation may serve to maintain calcium signaling functions, such as calcium-induced calcium release, it remains uncertain whether such compensation alleviates or contributes to the behavioral phenotype.

Leonard S. Dove - One of the best experts on this subject based on the ideXlab platform.

  • Homeostatic compensation maintains Ca2+ signaling functions in Purkinje neurons in the Leaner Mutant Mouse.
    Cerebellum (London England), 2002
    Co-Authors: David Murchison, Leonard S. Dove, Louise C. Abbott, William H. Griffith
    Abstract:

    Several human neurological disorders have been associated with mutations in the gene coding for the 1 subunit of the P/Q type voltage-gated calcium channel (α1A/CaV2.1). Mutations in this gene also occur in a number of neurolog-ically afflected Mouse strains, including Leaner (tgla/tgla). Because the P-type calcium current is very prominent in cerebellar Purkinje neurons, these cells from mice with α1 subunit mutations make excellent models for the investigation of the functional consequences of native mutations in a voltage-gated calcium channel of mammalian central nervous system. In this review, we describe the impact of altered channel function on cellular calcium homeostasis and signaling. Remarkably, calcium buffering functions of the endoplasmic reticulum and calcium-binding proteins appear to be regulated in order to compensate for altered calcium influx through the Mutant channels. Although this compensation may serve to maintain calcium signaling functions, such as calcium-induced calcium release, it remains uncertain whether such compensation alleviates or contributes to the behavioral phenotype.

  • Homeostatic compensation maintains Ca^2+ signaling functions in Purkinje neurons in the Leaner Mutant Mouse
    The Cerebellum, 2002
    Co-Authors: David Murchison, Leonard S. Dove, Louise C. Abbott, William H. Griffith
    Abstract:

    Several human neurological disorders have been associated with mutations in the gene coding for the 1 subunit of the P/Q type voltage-gated calcium channel (α1_A/Ca_V2.1). Mutations in this gene also occur in a number of neurolog-ically afflected Mouse strains, including Leaner (tg^la/tg^la). Because the P-type calcium current is very prominent in cerebellar Purkinje neurons, these cells from mice with α1 subunit mutations make excellent models for the investigation of the functional consequences of native mutations in a voltage-gated calcium channel of mammalian central nervous system. In this review, we describe the impact of altered channel function on cellular calcium homeostasis and signaling. Remarkably, calcium buffering functions of the endoplasmic reticulum and calcium-binding proteins appear to be regulated in order to compensate for altered calcium influx through the Mutant channels. Although this compensation may serve to maintain calcium signaling functions, such as calcium-induced calcium release, it remains uncertain whether such compensation alleviates or contributes to the behavioral phenotype.

Louise C. Abbott - One of the best experts on this subject based on the ideXlab platform.

  • Homeostatic compensation maintains Ca2+ signaling functions in Purkinje neurons in the Leaner Mutant Mouse.
    Cerebellum (London England), 2002
    Co-Authors: David Murchison, Leonard S. Dove, Louise C. Abbott, William H. Griffith
    Abstract:

    Several human neurological disorders have been associated with mutations in the gene coding for the 1 subunit of the P/Q type voltage-gated calcium channel (α1A/CaV2.1). Mutations in this gene also occur in a number of neurolog-ically afflected Mouse strains, including Leaner (tgla/tgla). Because the P-type calcium current is very prominent in cerebellar Purkinje neurons, these cells from mice with α1 subunit mutations make excellent models for the investigation of the functional consequences of native mutations in a voltage-gated calcium channel of mammalian central nervous system. In this review, we describe the impact of altered channel function on cellular calcium homeostasis and signaling. Remarkably, calcium buffering functions of the endoplasmic reticulum and calcium-binding proteins appear to be regulated in order to compensate for altered calcium influx through the Mutant channels. Although this compensation may serve to maintain calcium signaling functions, such as calcium-induced calcium release, it remains uncertain whether such compensation alleviates or contributes to the behavioral phenotype.

  • Homeostatic compensation maintains Ca^2+ signaling functions in Purkinje neurons in the Leaner Mutant Mouse
    The Cerebellum, 2002
    Co-Authors: David Murchison, Leonard S. Dove, Louise C. Abbott, William H. Griffith
    Abstract:

    Several human neurological disorders have been associated with mutations in the gene coding for the 1 subunit of the P/Q type voltage-gated calcium channel (α1_A/Ca_V2.1). Mutations in this gene also occur in a number of neurolog-ically afflected Mouse strains, including Leaner (tg^la/tg^la). Because the P-type calcium current is very prominent in cerebellar Purkinje neurons, these cells from mice with α1 subunit mutations make excellent models for the investigation of the functional consequences of native mutations in a voltage-gated calcium channel of mammalian central nervous system. In this review, we describe the impact of altered channel function on cellular calcium homeostasis and signaling. Remarkably, calcium buffering functions of the endoplasmic reticulum and calcium-binding proteins appear to be regulated in order to compensate for altered calcium influx through the Mutant channels. Although this compensation may serve to maintain calcium signaling functions, such as calcium-induced calcium release, it remains uncertain whether such compensation alleviates or contributes to the behavioral phenotype.

Firoze B. Jungalwala - One of the best experts on this subject based on the ideXlab platform.

  • Rostrocaudal expression of antibody HNK-1-reactive glycolipids in Mouse cerebellum: relationship to developmental compartments and Leaner mutation.
    The Journal of comparative neurology, 1993
    Co-Authors: S.m Nair, Nemani Prasadarao, Stuart A. Tobet, Firoze B. Jungalwala
    Abstract:

    Sulfoglucuronylglycolipids (SGGLs) and glycoproteins, reacting with monoclonal antibody HNK-1, are developmentally and spatially regulated in the mammalian cortex and cerebellum. It has been proposed that the HNK-1 carbohydrate epitope is involved in intercellular adhesion and cell-cell interactions. Biochemical analysis and immunocytochemical localization of SGGLs and other neolacto series glycolipids were studied in the Leaner Mutant Mouse cerebellum, where a slow and progressive rostral to caudal degeneration occurs with a gradual loss of both granule cells and Purkinje cells. Biochemical analyses showed that SGGLs and other neolacto series of glycolipids were significantly decreased in the adult Leaner cerebellum; however, HNK-1-reactive glycoproteins were not affected. By an immunocytochemical method which selectively localizes the lipid antigens, it is shown that SGGLs are primarily associated with Purkinje cell bodies and their dendrites in the molecular layer and in cerebellar nuclei where Purkinje cell axons terminate. At postnatal day 30 (P30), SGGL immunoreactivity (SGGL-ir) in the Leaner cerebellum was reduced moderately compared to normal littermates, which correlated with the minimal degree of Purkinje cell degeneration at this age in Leaner and with the biochemical data. At P67 and P90, the SGGL-ir was significantly more reduced in the Leaner as Purkinje cell degeneration proceeded. There was a direct correlation between loss of Purkinje cells and SGGL-ir in the cerebellar molecular layer. In both normal and young Leaner cerebella, the SGGL-ir in different lobules was not uniform; there were distinct rostrocaudal and mediolateral differences. SGGL-ir was markedly more intense in rostral than in caudal lobules in the vermis, the dividing line being the region immediately caudal to the primary fissure and rostral to the declival sulcus. In the lateral cerebellum, the SGGL-ir was less intense than in the vermis and the rostrocaudal difference was not as pronounced. There was also nonuniformity in the intensity of staining in different folia. The rostrocaudal as well as mediolateral differences in the intensity of SGGL-ir were confirmed independently by biochemical analysis. The differential phenotypic expression of SGGLs and the selective susceptibility to Purkinje cell death in Leaner Mutant are discussed in relation to the known embryologic and ontogenetic compartmentation of cerebellum.