BCS1L Chaperone necessary for the assembly of mitochondrial respiratory chain complex III. Plays an important role in the maintenance of mitochondrial tubular networks, respiratory chain assembly and formation of the LETM1 complex. Belongs to the AAA ATPase family. BCS1 subfamily. Ubiquitous. Note: This description may include information from UniProtKB.
Protein type: Chaperone; Membrane protein, integral; Mitochondrial
Chromosomal Location of Human Ortholog: 2q35
Cellular Component:  integral component of membrane; mitochondrial respiratory chain complex III; mitochondrion
Molecular Function:  ATP binding; protein binding
Biological Process:  mitochondrial respiratory chain complex I assembly; mitochondrial respiratory chain complex III assembly; mitochondrial respiratory chain complex IV assembly; mitochondrion organization
Disease: Bjornstad Syndrome; Gracile Syndrome; Leigh Syndrome; Mitochondrial Complex Iii Deficiency, Nuclear Type 1
Reference #:  Q9Y276 (UniProtKB)
Alt. Names/Synonyms: BCS; BCS1; BCS1-like; BCS1-like (yeast); BCS1-like protein; BCS1L; BJS; FLNMS; GRACILE; h-BCS; h-BCS1; Hs.6719; Mitochondrial chaperone BCS1; mitochondrial complex III assembly; PTD
Gene Symbols: BCS1L
Molecular weight: 47,534 Da
Basal Isoelectric point: 8.63  Predict pI for various phosphorylation states
Select Structure to View Below


Protein Structure Not Found.

Cross-references to other databases:  STRING  |  cBioPortal  |  Wikipedia  |  Reactome  |  neXtProt  |  Protein Atlas  |  BioGPS  |  Pfam  |  Phospho.ELM  |  NetworKIN  |  GeneCards  |  UniProtKB  |  Entrez-Gene  |  GenPept  |  Ensembl Gene