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Specialty: Biochemistry
Condition: Ataxia

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Total 9 results found since Jan 2013.

Reduction of fetuin-A levels contributes to impairment of Purkinje cells in cerebella of patients with Parkinson's disease
In conclusion, morphological abnormalities of Purkinje cells in PD mice and patients have a close relationship with a decrease of fetuin-A, suggesting that diagnosis and treatment of cerebellar functions of PD patients might be possible through regulation of fetuin-A.PMID:36935573
Source: BMB Reports - March 20, 2023 Category: Biochemistry Authors: Sunmi Yoon Napissara Boonpraman Chae Young Kim Jong-Seok Moon Sun Shin Yi Source Type: research

Chronic suppression of STIM1-mediated calcium signaling in Purkinje cells rescues the cerebellar pathology in spinocerebellar ataxia type 2
Biochim Biophys Acta Mol Cell Res. 2023 Mar 18:119466. doi: 10.1016/j.bbamcr.2023.119466. Online ahead of print.ABSTRACTDistorted neuronal calcium signaling has been reported in many neurodegenerative disorders, including different types of spinocerebellar ataxias (SCAs). Cerebellar Purkinje cells (PCs) are primarily affected in SCAs and the disturbances in the calcium homeostasis were observed in SCA PCs. Our previous results have revealed that 3,5-dihydroxyphenylglycine (DHPG) induced greater calcium responses in SCA2-58Q PC cultures than in wild type (WT) PC cultures. Here we observed that glutamate-induced calcium rele...
Source: Biochimica et Biophysica Acta - March 20, 2023 Category: Biochemistry Authors: Polina A Egorova Ksenia S Marinina Ilya B Bezprozvanny Source Type: research

Sez6l2 regulates phosphorylation of ADD and neuritogenesis.
Abstract Increasing evidence shows that immune-mediated mechanisms may contribute to the pathogenesis of central nervous system disorders including cerebellar ataxias, as indicated by the aberrant production of neuronal surface antibodies. We previously reported a patient with cerebellar ataxia associated with production of a new anti-neuronal antibody, anti-seizure-related 6 homolog like 2 (Sez6l2). Sez6l2 is a type 1 membrane protein that is highly expressed in the hippocampus and cerebellar cortex and mice lacking Sez6l2 protein family members develop ataxia. Here we used a proteomics-based approach to show tha...
Source: Biochemical and Biophysical Research communications - November 30, 2017 Category: Biochemistry Authors: Yaguchi H, Yabe I, Takahashi H, Watanabe M, Nomura T, Kano T, Matsumoto M, Nakayama KI, Watanabe M, Hatakeyama S Tags: Biochem Biophys Res Commun Source Type: research

Neurotoxic mechanisms by which the USP14 inhibitor IU1 depletes ubiquitinated proteins and Tau in rat cerebral cortical neurons: relevance to Alzheimer's disease.
In conclusion, pharmacologically inhibiting (with low or high IU1 concentrations) or genetically down-regulating USP14 fail to enhance proteasomal degradation of Ub-proteins or Tau in neurons. PMID: 28372990 [PubMed - as supplied by publisher]
Source: Biochimica et Biophysica Acta - March 31, 2017 Category: Biochemistry Authors: Kiprowska MJ, Stepanova A, Todaro DR, Galkin A, Haas A, Wilson SM, Figueiredo-Pereira ME Tags: Biochim Biophys Acta Source Type: research

Ataxia telangiectasia mutated inhibits oxidative stress-induced apoptosis by regulating heme oxygenase-1 expression.
In conclusion, ATM induces HO-1 expression via activation of PKC-δ and NF-κB and inhibits oxidative stress-induced apoptosis. A loss of HO-1 induction may explain why AT patients are vulnerable to oxidative stress. PMID: 25592228 [PubMed - as supplied by publisher]
Source: The International Journal of Biochemistry and Cell Biology - January 12, 2015 Category: Biochemistry Authors: Yu JH, Cho SO, Lim JW, Kim N, Kim H Tags: Int J Biochem Cell Biol Source Type: research

Mutant γPKC that causes spinocerebellar ataxia type 14 upregulates Hsp70, which protects cells from the mutant's cytotoxicity.
Abstract Several missense mutations in the protein kinase Cγ (γPKC) gene have been found to cause spinocerebellar ataxia type 14 (SCA14), an autosomal dominant neurodegenerative disease. We previously demonstrated that the mutant γPKC found in SCA14 is misfolded, susceptible to aggregation and cytotoxic. Molecular chaperones assist the refolding and degradation of misfolded proteins and prevention of the proteins' aggregation. In the present study, we found that the expression of mutant γPKC-GFP increased the levels of heat-shock protein 70 (Hsp70) in SH-SY5Y cells. To elucidate the role of this elevation, we ...
Source: Biochemical and Biophysical Research communications - September 7, 2013 Category: Biochemistry Authors: Ogawa K, Seki T, Onji T, Adachi N, Tanaka S, Hide I, Saito N, Sakai N Tags: Biochem Biophys Res Commun Source Type: research