HCCS
HCCS (holocytochrome c synthase, also known as CCHL or MLS) is a mitochondrial inner membrane lyase that covalently attaches heme to apocytochrome c, producing the mature, functional cytochrome c. It belongs to the cytochrome c-type heme lyase family. Browse Assay Genie's current range of HCCS research reagents below, or read on for the biology behind the target.
HCCS is a lyase that catalyses the covalent linking of the heme group to the cytochrome c apoprotein. This reaction converts the apoprotein into mature, functional cytochrome c. The enzyme is classified as EC 4.4.1.17 and belongs to the cytochrome c-type heme lyase family. Its UniProt name is holocytochrome c-type synthase, and an earlier HGNC name describes it as cytochrome c heme-lyase.
UniProt places the HCCS protein in the mitochondrion inner membrane, where cytochrome c maturation takes place. The HCCS gene is listed by HGNC within the group of mitochondrial respiratory chain complex assembly factors. The mouse ortholog is Hccs. According to the NCBI Gene summary, three transcript variants have been found for the gene, and all of them encode the same protein.
The gene has also been known by the symbols CCHL and MLS. MLS comes from the earlier disease-based name, microphthalmia with linear skin defects. Defects in HCCS are a cause of microphthalmia syndromic type 7 (MCOPS7). This link between a heme-attachment enzyme and a developmental syndrome makes HCCS a focus for work on mitochondrial biology and cytochrome c maturation.
HCCS attaches heme to apocytochrome c, so it is the enzyme to study when examining how functional cytochrome c is produced.
As an inner membrane enzyme listed among respiratory chain complex assembly factors, HCCS is relevant to studies of mitochondrial function.
Defects in HCCS cause microphthalmia syndromic type 7 (MCOPS7), making it a target in research on this developmental disorder.
HCCS is a cytochrome c-type heme lyase, EC 4.4.1.17, and serves as a model for covalent heme attachment.
HCCS is a lyase that catalyses the covalent linking of a heme group to the cytochrome c apoprotein. This produces the mature, functional cytochrome c. The enzyme sits in the mitochondrion inner membrane and is classified as EC 4.4.1.17.
HCCS is also known as CCHL and MLS. Its previous names are holocytochrome c synthase (cytochrome c heme-lyase) and microphthalmia with linear skin defects. UniProt calls the protein holocytochrome c-type synthase. NCBI reports three transcript variants that encode the same protein.
Defects in HCCS are a cause of microphthalmia syndromic type 7 (MCOPS7). The gene has a mouse ortholog, Hccs, so mouse studies can address its function. Check the datasheet of any product for its validated reactivity.
Other cytochrome c pathway proteins, mitochondrial respiratory chain assembly factors and heme-related targets with products at Assay Genie.
Gene and protein annotations from HGNC, NCBI Gene and UniProt P53701; product specifications from Assay Genie datasheets.