MIA40 Blocking Peptide | CHCHD4 blocking peptide

MIA40 Antibody (C-term) Blocking peptide

• Gene Names: CHCHD4; MIA40; TIMM40
• Synonyms: MIA40; MIA40 Antibody (C-term) Blocking peptide; Mitochondrial intermembrane space import and assembly protein 40; Coiled-coil-helix-coiled-coil-helix domain-containing protein 4; CHCHD4; CHCHD4 blocking peptide

• Form/Format: Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.
• Sequence Length: 142

• Cellular Location: Mitochondrion intermembrane space
• Tissue Location: Expressed in all tissues tested, suggesting an ubiquitous expression.
• Preparation and Storage: Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C.

Related Product Information for CHCHD4 blocking peptide

Functions as chaperone and catalyzes the formation of disulfide bonds in substrate proteins, such as COX17. Required for the import and folding of small cysteine-containing proteins (small Tim) in the mitochondrial intermembrane space (IMS). Precursor proteins to be imported into the IMS are translocated in their reduced form into the mitochondria. The oxidized form of CHCHD4/MIA40 forms a transient intermolecular disulfide bridge with the reduced precursor protein, resulting in oxidation of the precursor protein that now contains an intramolecular disulfide bond and is able to undergo folding in the IMS. Reduced CHCHD4/MIA40 is then reoxidized by GFER/ERV1 via a disulfide relay system.

NCBI and Uniprot Product Information

• NCBI GI #: 62510498
• NCBI GeneID: 131474
• UniProt Accession #: Q8N4Q1; Q96AI2; Q96MY6; A8K3Z9
• Molecular Weight: 17,385 Da
• NCBI Official Full Name: Mitochondrial intermembrane space import and assembly protein 40
• NCBI Official Synonym Full Names: coiled-coil-helix-coiled-coil-helix domain containing 4
• NCBI Official Symbol: CHCHD4
• NCBI Official Synonym Symbols: MIA40; TIMM40
• NCBI Protein Information: mitochondrial intermembrane space import and assembly protein 40
• UniProt Protein Name: Mitochondrial intermembrane space import and assembly protein 40
• Protein Family: Mitochondrial intermembrane space import and assembly protein
• UniProt Gene Name: CHCHD4
• UniProt Synonym Gene Names: MIA40
• UniProt Entry Name: MIA40_HUMAN

NCBI Description

CHCHD4, a component of human mitochondria, belongs to a protein family whose members share 6 highly conserved cysteine residues constituting a -CXC-CX(9)C-CX(9)C- motif in the C terminus (Hofmann et al., 2005 [PubMed 16185709]).[supplied by OMIM, Mar 2008]

Uniprot Description

CHCHD4: Required for the import and folding of small cysteine- containing proteins (small Tim) in the mitochondrial intermembrane space (IMS). Probably acts by forming a redox cycle with GFER/ERV1 that involves a disulfide relay system. Precursor proteins to be imported into the IMS are translocated in their reduced form into the mitochondria. The oxidized form of MIA40 forms a transient intermolecular disulfide bridge with the reduced precursor protein, resulting in oxidation of the precursor protein that now contains an intramolecular disulfide bond and is able to undergo folding in the IMS (Probable). 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Mitochondrial

Chromosomal Location of Human Ortholog: 3p25.1

Cellular Component: mitochondrial intermembrane space; mitochondrion

Molecular Function: protein disulfide oxidoreductase activity

Biological Process: 'de novo' posttranslational protein folding; protein import into mitochondrial intermembrane space; protein maturation via protein folding