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GUF1
GUF1 (GTP binding elongation factor GUF1) is a mitochondrial translational GTPase that promotes protein synthesis in the organelle. It is also known as FLJ13220 and was previously named GUF1 GTPase homolog (S. cerevisiae). Browse Assay Genie's current range of GUF1 research reagents below, or read on for the biology behind the target.
GUF1 promotes mitochondrial protein synthesis. It is thought to act as a fidelity factor in the translation reaction, catalysing a one-codon backward translocation of tRNAs on ribosomes that have been improperly translocated. The NCBI Gene summary describes it as a GTPase that triggers back-translocation of the elongating ribosome, and suggests it helps prevent amino acid misincorporation under stressful, suboptimal conditions. GUF1 binds to mitochondrial ribosomes in a GTP-dependent manner.
The GUF1 protein localises to the mitochondrion inner membrane. It belongs to the TRAFAC class translation factor GTPase superfamily, within the classic translation factor GTPase family and the LepA subfamily. HGNC places the GUF1 gene in the translational GTPases group. The protein contains a highly conserved C-terminal domain that is not found in other GTPases and that facilitates tRNA binding. Alternative splicing of the gene results in multiple transcript variants. A mouse ortholog, Guf1, is recorded.
The name reflects the yeast homolog: earlier HGNC names were GUF1 GTPase homolog (S. cerevisiae) and GUF1 homolog, GTPase. An allelic variant of the GUF1 gene has been associated with early infantile epileptic encephalopathy-40, which links mitochondrial translation quality control to human disease.
GUF1 promotes mitochondrial protein synthesis and binds mitochondrial ribosomes in a GTP-dependent manner, making it a useful subject for studying organellar translation.
GUF1 may catalyse one-codon backward tRNA translocation on improperly translocated ribosomes, a proposed mechanism for limiting amino acid misincorporation.
The protein is thought to prevent misincorporation of amino acids in stressful, suboptimal conditions, relevant to studies of mitochondrial stress.
An allelic variant of GUF1 has been associated with early infantile epileptic encephalopathy-40, linking the gene to inherited neurological disorders.
GUF1 promotes mitochondrial protein synthesis. It may act as a fidelity factor, catalysing a one-codon backward translocation of tRNAs on improperly translocated ribosomes, and it binds mitochondrial ribosomes in a GTP-dependent manner. It is thought to limit amino acid misincorporation under suboptimal conditions.
HGNC names the gene GTP binding elongation factor GUF1, and UniProt lists the protein as translation factor GUF1, mitochondrial. Other names are FLJ13220, GUF1 GTPase homolog (S. cerevisiae) and GUF1 homolog, GTPase. Alternative splicing produces multiple transcript variants.
GUF1 has a mouse ortholog, Guf1, and a yeast homolog that gave the gene its earlier names. Its C-terminal domain is highly conserved and absent from other GTPases. An allelic variant has been associated with early infantile epileptic encephalopathy-40.
Other translational GTPases and mitochondrial translation factors with products at Assay Genie.
Gene and protein annotations from HGNC, NCBI Gene and UniProt Q8N442; product specifications from Assay Genie datasheets.
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