The protein encoded by this gene is a mitochondrial protein with two putative domains, an N-terminal mitochondrial localization sequence, and a UPF0099 domain. In vitro assays suggest that this protein possesses peptidyl-tRNA hydrolase activity, to release the peptidyl moiety from tRNA, thereby preventing the accumulation of dissociated peptidyl-tRNA that could reduce the efficiency of translation. This protein also plays a role regulating cell survival and death. It promotes survival as part of an integrin-signaling pathway for cells attached to the extracellular matrix (ECM), but also promotes apoptosis in cells that have lost their attachment to the ECM, a process called anoikos. After loss of cell attachment to the ECM, this protein is phosphorylated, is released from the mitochondria into the cytosol, and promotes caspase-independent apoptosis through interactions with transcriptional regulators. This gene has been implicated in the development and progression of tumors, and mutations in this gene have been associated with an infantile multisystem neurologic, endocrine, and pancreatic disease (INMEPD) characterized by intellectual disability, postnatal microcephaly, progressive cerebellar atrophy, hearing impairment, polyneuropathy, failure to thrive, and organ fibrosis with exocrine pancreas insufficiency (PMID: 25574476). Alteative splicing results in multiple transcript variants encoding different isoforms.
Categories
Primary Antibodies
Clonality
polyclonal
Description
The natural substrate for this enzyme may be peptidyl- tRNAs which drop off the ribosome during protein synthesis. Promotes caspase-independent apoptosis by regulating the function of two transcriptional regulators, AES and TLE1.
Host
Rabbit
Immunogen
peptidyl-tRNA hydrolase 2
Isotype
IgG
Molecular Weight
20 kDa
Reactivity
Human, Mouse, Rat
Regulatory
RUO
Synonyms
BIT1, PTH2
Uniprot
Q9Y3E5
Gene Id
51651
Research Area
Metabolism
Form
liquid
Format
liquid
Purification
Immunogen affinity purified
Purity
>=95% as determined by SDS-PAGE
Storage
PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20°C for 12 months (Avoid repeated freeze / thaw cycles.)