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1993 2
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[Complex V deficiency].
Matsumoto H, Nishino I. Matsumoto H, et al. Nihon Rinsho. 2002 Apr;60 Suppl 4:495-8. Nihon Rinsho. 2002. PMID: 12013922 Review. Japanese. No abstract available.
Mitochondrial encephalomyopathies: an update.
DiMauro S, Hirano M. DiMauro S, et al. Neuromuscul Disord. 2005 Apr;15(4):276-86. doi: 10.1016/j.nmd.2004.12.008. Neuromuscul Disord. 2005. PMID: 15792866 Review.
Advances in mendelian mitochondrial diseases include: (i) new mutations in genes for complex I subunits; (ii) identification of new mutant ancillary proteins associated with complex IV and complex V deficiencies; (iii) better molecular understanding of disord …
Advances in mendelian mitochondrial diseases include: (i) new mutations in genes for complex I subunits; (ii) identification of new mutant a …
Mitochondrial ATP synthase: architecture, function and pathology.
Jonckheere AI, Smeitink JA, Rodenburg RJ. Jonckheere AI, et al. J Inherit Metab Dis. 2012 Mar;35(2):211-25. doi: 10.1007/s10545-011-9382-9. Epub 2011 Aug 27. J Inherit Metab Dis. 2012. PMID: 21874297 Free PMC article. Review.
This review covers the architecture, function and assembly of complex V. The role of complex V di-and oligomerization and its relation with mitochondrial morphology is discussed. Finally, pathology related to complex V deficiency a …
This review covers the architecture, function and assembly of complex V. The role of complex V di-and oligomeriz …
Mitochondrial acetylation and genetic models of Parkinson's disease.
Auburger G, Gispert S, Jendrach M. Auburger G, et al. Prog Mol Biol Transl Sci. 2014;127:155-82. doi: 10.1016/B978-0-12-394625-6.00006-4. Prog Mol Biol Transl Sci. 2014. PMID: 25149217 Review.
This mitophagy process is closely regulated by PINK1 and Parkin, two interacting proteins which relocalize to mitochondria with deficient proton gradients, and whose mutations cause autosomal recessive variants of PD. ...
This mitophagy process is closely regulated by PINK1 and Parkin, two interacting proteins which relocalize to mitochondria with deficient …
Lacticacidemia.
Robinson BH. Robinson BH. Biochim Biophys Acta. 1993 Oct 20;1182(3):231-44. doi: 10.1016/0925-4439(93)90064-8. Biochim Biophys Acta. 1993. PMID: 8399357 Review. No abstract available.
The expanding clinical spectrum of mitochondrial diseases.
De Vivo DC. De Vivo DC. Brain Dev. 1993 Jan-Feb;15(1):1-22. doi: 10.1016/0387-7604(93)90002-p. Brain Dev. 1993. PMID: 8338207 Review.
Disturbances of pyruvate or fatty acid metabolism are examples of substrate utilization defects. Only four defects of the Krebs cycle are known: fumarase deficiency, dihydrolipoyl dehydrogenase deficiency, alpha-ketoglutarate dehydrogenase deficiency, and com …
Disturbances of pyruvate or fatty acid metabolism are examples of substrate utilization defects. Only four defects of the Krebs cycle are kn …
MtDNA and nuclear mutations affecting oxidative phosphorylation: correlating severity of clinical defect with extent of bioenergetic compromise.
Robinson BH. Robinson BH. J Bioenerg Biomembr. 1994 Jun;26(3):311-6. doi: 10.1007/BF00763102. J Bioenerg Biomembr. 1994. PMID: 8077184 Review.
Rates of ATP synthesis were studied in cultured skin fibroblasts treated with digitonin. In fibroblasts from patients with complex I deficiency, complex IV and complex V deficiency rates of ATP synthesis were decreased below the levels found in control …
Rates of ATP synthesis were studied in cultured skin fibroblasts treated with digitonin. In fibroblasts from patients with complex I defi …
Variants in Human ATP Synthase Mitochondrial Genes: Biochemical Dysfunctions, Associated Diseases, and Therapies.
Del Dotto V, Musiani F, Baracca A, Solaini G. Del Dotto V, et al. Int J Mol Sci. 2024 Feb 13;25(4):2239. doi: 10.3390/ijms25042239. Int J Mol Sci. 2024. PMID: 38396915 Free PMC article. Review.
Mitochondrial ATP synthase (Complex V) catalyzes the last step of oxidative phosphorylation and provides most of the energy (ATP) required by human cells. The mitochondrial genes MT-ATP6 and MT-ATP8 encode two subunits of the multi-subunit Complex V. S …
Mitochondrial ATP synthase (Complex V) catalyzes the last step of oxidative phosphorylation and provides most of the energy (A …
[Complex V (ATP synthase) deficiency].
Nonaka I. Nonaka I. Ryoikibetsu Shokogun Shirizu. 2001;(36):142-3. Ryoikibetsu Shokogun Shirizu. 2001. PMID: 11596347 Review. Japanese. No abstract available.
[Application of new techniques for locating the underlying molecular defects in patients with disorders of oxidative phosphorylation].
Van Coster R, Smet J. Van Coster R, et al. Verh K Acad Geneeskd Belg. 2007;69(4):197-211. Verh K Acad Geneeskd Belg. 2007. PMID: 17821958 Review. Dutch.
BN-PAGE followed by activity staining in the gel is especially suited for detection of a deficiency of complex I or of complex V With the spectrophotometric method is it difficult to detect these deficiencies in cultured skin fibroblasts. With BN-PAGE …
BN-PAGE followed by activity staining in the gel is especially suited for detection of a deficiency of complex I or of complex …