CHCHD3
CHCHD3 (coiled-coil-helix-coiled-coil-helix domain containing 3, also known as MIC19, MINOS3 or PPP1R22) is a scaffold protein of the mitochondrial inner membrane. It is a subunit of the MICOS complex and helps maintain cristae architecture. Browse Assay Genie's current range of CHCHD3 research reagents below, or read on for the biology behind the target.
CHCHD3 is a component of the MICOS complex, a large protein assembly in the mitochondrial inner membrane. MICOS maintains crista junctions, shapes inner membrane architecture and forms contact sites with the outer membrane. CHCHD3 contributes to the stability of the complex and to cristae morphology. NCBI Gene describes the protein as an inner mitochondrial membrane scaffold protein, and loss of it affects the structural integrity of cristae. This is linked to reduced ATP production, slower cell growth and lower oxygen consumption.
UniProt also reports a second role for CHCHD3. It has been shown to act as a transcription factor that binds the BAG1 promoter and represses BAG1 transcription. This fits the annotated locations, which include the mitochondrion inner membrane, the cytoplasm and the nucleus. The CHCHD3 protein belongs to the MICOS complex subunit Mic19 family, in the metazoan Mic19 subfamily. Several transcript variants encoding different isoforms of the CHCHD3 gene have been described.
The HGNC name refers to the coiled-coil-helix-coiled-coil-helix domain. The gene is placed in several HGNC groups: CHCH domain containing proteins, protein phosphatase 1 regulatory subunits, MICOS complex subunits and microRNA protein coding host genes. Aliases include FLJ20420, MINOS3, PPP1R22, Mic19 and MICOS19. The mouse ortholog is Chchd3. Related CHCH domain proteins, such as CHCHD2, are often studied alongside it in mitochondrial biology.
CHCHD3 helps maintain crista junctions and cristae morphology, so it is a useful subject for studies of inner membrane architecture.
As a MICOS subunit that supports complex stability, CHCHD3 is relevant to work on contact sites between the inner and outer mitochondrial membranes.
Absence of CHCHD3 reduces ATP production and oxygen consumption, linking cristae structure to mitochondrial function and cell growth.
CHCHD3 has been shown to bind the BAG1 promoter and repress BAG1 transcription, pointing to a role beyond the mitochondrion.
CHCHD3 is a subunit of the MICOS complex in the mitochondrial inner membrane. It supports complex stability, crista junctions and cristae morphology. UniProt also reports that it can bind the BAG1 promoter and repress BAG1 transcription.
CHCHD3 is also called MIC19, MICOS19, MINOS3, PPP1R22 and FLJ20420. UniProt names the protein MICOS complex subunit MIC19. NCBI Gene notes that several transcript variants encode different isoforms.
A mouse ortholog, Chchd3, is recorded. The protein belongs to the MICOS complex subunit Mic19 family, in the metazoan Mic19 subfamily. Check the datasheet of any product for its validated reactivity.
Other coiled-coil-helix-coiled-coil-helix domain proteins and mitochondrial targets with products at Assay Genie.
Gene and protein annotations from HGNC, NCBI Gene and UniProt Q9NX63; product specifications from Assay Genie datasheets.