Deficiency of the Voltage-Dependent Anion Channel: A Novel Cause of Mitochondriopathy
نویسندگان
چکیده
منابع مشابه
wuthering heights and the concept of marality/a sociological study of the novel
to discuss my point, i have collected quite a number of articles, anthologies, and books about "wuthering heights" applying various ideas and theories to this fantastic story. hence, i have come to believe that gadamer and jauss are rightful when they claim that "the individaul human mind is the center and origin of all meaning," 3 that reading literature is a reader-oriented activity, that it ...
15 صفحه اولA 3D model of the voltage-dependent anion channel (VDAC).
Eukaryotic porins are a group of membrane proteins whose best known role is to form an aqueous pore channel in the mitochondrial outer membrane. As opposed to the bacterial porins (a large family of protein whose 3D structure has been determined by X-ray diffraction), the structure of eukaryotic porins (also termed VDACs, voltage-dependent anion-selective channels) is still a matter of debate. ...
متن کاملStructure of the human voltage-dependent anion channel.
The voltage-dependent anion channel (VDAC), also known as mitochondrial porin, is the most abundant protein in the mitochondrial outer membrane (MOM). VDAC is the channel known to guide the metabolic flux across the MOM and plays a key role in mitochondrially induced apoptosis. Here, we present the 3D structure of human VDAC1, which was solved conjointly by NMR spectroscopy and x-ray crystallog...
متن کاملMonika voltage - dependent anion channel Nucleotide interactions of the human
The voltage-dependent anion channel (VDAC) mediates and gates the flux of metabolites and ions across the outer mitochondrial membrane (OMM) and is a key player in cellular metabolism and apoptosis. Here we characterized the binding of nucleotides to human VDAC1 (hVDAC1) on a single-residue level using NMR spectroscopy and site-directed mutagenesis. We find that hVDAC1 possesses one major bindi...
متن کاملLack of voltage-dependent anion channel in human mitochondrial myopathies.
SIR—In several hundreds of patients with a mitochondrial myopathy an enzyme defect in mitochondrial energy metabolism is identified. However, in a substantial number of subjects no enzyme defect can be detected, although diminished substrate oxidation and ATP production rates are found in their muscle in vitro. The hypothesis, that in this group of patients proteins for transport of vario...
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ژورنال
عنوان ژورنال: Pediatric Research
سال: 1996
ISSN: 0031-3998,1530-0447
DOI: 10.1203/00006450-199605000-00003