Description: Homo sapiens coenzyme Q4 (COQ4), transcript variant 1, mRNA; nuclear gene for mitochondrial product. (from RefSeq NM_016035) RefSeq Summary (NM_016035): This gene encodes a component of the coenzyme Q biosynthesis pathway. Coenzyme Q, an essential component of the electron transport chain, shuttles electrons between complexes I or II to complex III of the mitochondrial transport chain. This protein appears to play a structural role in stabilizing a complex that contains most of the coenzyme Q biosynthesis enzymes. Mutations in this gene are associated with mitochondrial disorders linked to coenzyme Q deficiency. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Apr 2015]. Gencode Transcript: ENST00000300452.8 Gencode Gene: ENSG00000167113.11 Transcript (Including UTRs) Position: hg38 chr9:128,322,839-128,334,072 Size: 11,234 Total Exon Count: 7 Strand: + Coding Region Position: hg38 chr9:128,322,859-128,333,645 Size: 10,787 Coding Exon Count: 7
ID:COQ4_HUMAN DESCRIPTION: RecName: Full=Ubiquinone biosynthesis protein COQ4 homolog, mitochondrial; AltName: Full=Coenzyme Q biosynthesis protein 4 homolog; Flags: Precursor; FUNCTION: Component of the coenzyme Q biosynthetic pathway. May play a role in organizing a multi-subunit COQ enzyme complex required for coenzyme Q biosynthesis. Required for steady-state levels of other COQ polypeptides. PATHWAY: Cofactor biosynthesis; ubiquinone biosynthesis. SUBUNIT: Component of a multi-subunit COQ enzyme complex, composed of at least COQ3, COQ4, COQ5, COQ6, COQ7 and COQ9 (By similarity). SUBCELLULAR LOCATION: Isoform 1: Mitochondrion inner membrane; Peripheral membrane protein; Matrix side. TISSUE SPECIFICITY: Expressed ubiquitously, but at high levels in liver, lung and pancreas. SIMILARITY: Belongs to the COQ4 family.
The RNAfold program from the Vienna RNA Package is used to perform the secondary structure predictions and folding calculations. The estimated folding energy is in kcal/mol. The more negative the energy, the more secondary structure the RNA is likely to have.
Pfam Domains: PF05019 - Coenzyme Q (ubiquinone) biosynthesis protein Coq4
ModBase Predicted Comparative 3D Structure on Q9Y3A0
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Orthologous Genes in Other Species
Orthologies between human, mouse, and rat are computed by taking the best BLASTP hit, and filtering out non-syntenic hits. For more distant species reciprocal-best BLASTP hits are used. Note that the absence of an ortholog in the table below may reflect incomplete annotations in the other species rather than a true absence of the orthologous gene.