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cDNA sequence and chromosomal localization of the NDUFS8 human gene coding for the 23 kDa subunit of the mitochondrial complex I.
Laboratoire BECP/EA 2019 UJF-DBMS, CEA Grenoble, France. procaccio@homme.ceng.cea.fr
We have sequenced the cDNA for the 23 kDa subunit of the human mitochondrial respiratory complex I. The deduced protein consists of 210 amino acids (Mr = 23705 Da) with a 34 amino acid N terminus presumably acting as a presequence for mitochondrial import. The predicted mature protein (Mr = 20290 Da) is 92% identical to the bovine mitochondrial subunit and 72% to the Rhodobacter capsulatus NUOI counterpart. Two clusters of four cysteine residues are conserved among these proteins. The gene (NDUFS8) coding for the human subunit has been mapped to chromosome 11q13.
PMID: 9116042 [PubMed - indexed for MEDLINE]
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Cited by 2 PubMed Central articles
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Respiratory chain complex I is essential for sexual development in neurospora and binding of iron sulfur clusters are required for enzyme assembly.
Duarte M, Videira A.
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[Genetics. 2000]
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The first nuclear-encoded complex I mutation in a patient with Leigh syndrome.
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[Am J Hum Genet. 1998]