HscB is a 235 amino acid mitochondrial protein that functions as a co-chaperone in iron-sulfur cluster formation. Highly expressed in heart, liver and muscle, and belonging to the HscB family, HscB exists as a L-shaped crystal structure resembling E. coli HscB. Human HscB contains an N-terminal mitochondrial targeting signal followed by a J-domain and short loop. The SLCterminal domain folds into a compact 3-helix bundle and likely mediates specific interactions with IscU. Containing six exons and five introns, the gene encoding HscB maps to human chromosome 22, which houses over 500 genes and is the second smallest human chromosome.
Function:
May act as a co-chaperone in iron-sulfur cluster assembly in mitochondria.
Subcellular Location:
Mitochondrion.
Tissue Specificity:
Liver, muscle and heart.
Similarity:
Belongs to the hscB family.
Contains 1 J domain.
SWISS:
Q8K3A0
Gene ID:
100900
Database links:
Entrez Gene: 150274 Human
Entrez Gene: 100900 Mouse
Omim: 608142 Human
SwissProt: Q8IWL3 Human
SwissProt: Q8K3A0 Mouse
Unigene: 632780 Human
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