Recombinant Human Iron-sulfur cluster co-chaperone protein HscB, mitochondrial (HSCB)

Uniprot Id

Q8IWL3

Target Species

Human

Target Name

HSCB

Target Full Name

Iron-sulfur cluster co-chaperone protein HscB

Target Function

Acts as a co-chaperone in iron-sulfur cluster assembly in both mitochondria and the cytoplasm. Required for incorporation of iron-sulfur clusters into SDHB, the iron-sulfur protein subunit of succinate dehydrogenase that is involved in complex II of the mitochondrial electron transport chain. Recruited to SDHB by interaction with SDHAF1 which first binds SDHB and then recruits the iron-sulfur transfer complex formed by HSC20, HSPA9 and ISCU through direct binding to HSC20. Also mediates complex formation between components of the cytosolic iron-sulfur biogenesis pathway and the CIA targeting complex composed of CIAO1, DIPK1B/FAM69B and MMS19 by binding directly to the scaffold protein ISCU and to CIAO1. This facilitates iron-sulfur cluster insertion into a number of cytoplasmic and nuclear proteins including POLD1, ELP3, DPYD and PPAT.

Target Subcellular Location

[Iron-sulfur cluster co-chaperone protein HscB, cytoplasmic]: Cytoplasm.; [Iron-sulfur cluster co-chaperone protein HscB, mitochondrial]: Mitochondrion.

Target Protein Families

HscB family

Target Tissue Specificity

Expressed in lung, brain, stomach, spleen, ovary, testis, liver, muscle and heart.

Target Synonyms

AI325508; AW049829; dJ366L4.2; DnaJ (Hsp40) homolog, subfamily C member 20; DnaJ homolog subfamily C member 20; DNAJC20; Hsc20; HSC20_HUMAN; hscB; HscB iron sulfur cluster co chaperone homolog; Iron sulfur cluster co chaperone protein HscB mitochondrial; Iron-sulfur cluster co-chaperone protein HscB; J type co chaperone HSC20; JAC1; mitochondrial; RGD1311005; RP3-366L4.2

Target Background

This gene encodes a DnaJ-type co-chaperone and member of the heat shock cognate B (HscB) family of proteins. The encoded protein plays a role in the synthesis of iron-sulfur clusters, protein cofactors that are involved in the redox reactions of mitochondrial electron transport and other processes. Cells in which this gene is knocked down exhibit reduced activity of iron-sulfur cluster-dependent enzymes including succinate dehydrogenase and aconitase. The encoded protein may stimulate the ATPase activity of the mitochondrial stress-70 protein. Alternative splicing results in multiple transcript variants.