产品名称
MTU1 Rabbit Polyclonal Antibody
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
https://www.uniprot.org/uniprot/55687
Human Swissprot No.
O75648
Human Swissprot Link
https://www.uniprot.org/uniprotkb/O75648/entry
Mouse Gene Link
https://www.uniprot.org/uniprot/72026
Mouse Swissprot No.
Q9DAT5
Mouse Swissprot Link
https://www.uniprot.org/uniprotkb/Q9DAT5
Rat Swissprot Link
https://www.uniprot.org/uniprotkb/B1WC37
免疫原
Synthesized peptide derived from human MTU1 AA range: 368-418
特异性
This antibody detects endogenous levels of MTU1 at Human/Mouse/Rat
宿主
Polyclonal, Rabbit,IgG
背景介绍
This nuclear gene encodes a mitochondrial tRNA-modifying enzyme. The encoded protein catalyzes the 2-thiolation of uridine on the wobble positions of tRNA(Lys), tRNA(Glu), and tRNA(Gln), resulting in the formation of 5-taurinomethyl-2-thiouridine moieties. Mutations in this gene may cause transient infantile liver failure. Polymorphisms in this gene may also influence the severity of deafness caused by mitochondrial 12S ribosomal RNA mutations. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Sep 2013],
组织表达
Ubiquitous. Abundantly expressed in tissues with high metabolic rates including heart, liver, kidney, and brain.
功能
caution:Was originally (PubMed:16513084) thought to be a 5-methylaminomethyl-2-methyltransferase involved in tRNA modification.,function:Catalyzes the 2-thiolation of uridine at the wobble position (U34) of mitochondrial tRNA(Lys), tRNA(Glu) and tRNA(Gln). Required for the formation of 5-taurinomethyl-2-thiouridine (tm5s2U) of mitochondrial tRNA(Lys), tRNA(Glu), and tRNA(Gln) at the wobble position. ATP is required to activate the C2 atom of the wobble base.,miscellaneous:During the reaction, ATP is used to activate the C2 atom of U34 by adenylation. After this, the persulfide sulfur on the catalytic cysteine is transferred to the C2 atom of the wobble base (U34) of mitochondrial tRNA(Lys), tRNA(Glu) and tRNA(Gln). The reaction probably involves hydrogen sulfide that is generated from the persulfide intermediate and that acts as nucleophile towards the activated C2 atom on U34. Subsequently, a transient disulfide bond is formed between the two active site cysteine residues.,polymorphism:The polymorphism Ser-10 aggravates the mitochondrial dysfunction associated with a mitochondrial 12S ribosomal RNA (rRNA) A1555G mutation that has been associated with aminoglycoside-induced and non-syndromic deafness [MIM:580000].,similarity:Belongs to the mnmA/TRMU family.,tissue specificity:Ubiquitous. Abundantly expressed in tissues with high metabolic rates including heart, liver, kidney, and brain.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
