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This locus encodes a GTP-binding protein. 再加上，我们可以发GTPBP3 抗体 (35) 和 GTPBP3 试剂盒 (6)和数多这个蛋白质的别的产品。
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defective expression of gtpbp3 related to tRNA modification alters the mitochondrial function and development of zebrafish
These observations suggest that the mouse Gtpbp3 is an evolutionarily conserved mitochondrial GTP-binding protein involved in the tRNA modification.
GTPBP3 defective expression is associated with an mitochondrial-tRNA hypomodification status.GTPBP3 plays a role in the regulation of UCP2 (显示 UCP2 蛋白) and MCP1 (显示 CCL2 蛋白) proteins through AMPK (显示 PRKAA1 蛋白) signaling.
Results show that post-transcriptional expression of GTPBP3, MTO1 and TRMU (显示 TRMU 蛋白) genes is down-regulated, leading to mt-tRNA hypomodification and contributing to mitochondrial dysfunction in MELAS cybrids.
Most individuals with GTPBP3 mutations developed neurological symptoms and MRI (显示 C7ORF49 蛋白) involvement of thalamus, putamen, and brainstem resembling Leigh syndrome.
GTPBP3 localizes in the mitochondria and is a deafness-associated homolog of yeast MSS1.
Phenotype of non-syndromic deafness associated with the mitochondrial A1555G mutation is modulated by mitochondrial RNA modifying enzymes MTO1 and GTPBP3.
Data show that the two most abundant GTPBP3 isoforms exhibit moderate affinity for guanine nucleotides like their bacterial homologue, MnmE, although they hydrolyze GTP (显示 AK3 蛋白) at a 100-fold lower rate.
This locus encodes a GTP-binding protein. The encoded protein is localized to the mitochondria and may play a role in mitochondrial tRNA modification. Polymorphisms at this locus may be associated with severity of aminoglycoside-induced deafness, a disease associated with a mutation in the 12S rRNA. Alternatively spliced transcript variants encoding different isoforms have been described.
GTP binding protein 3 (mitochondrial)
, tRNA modification GTPase GTPBP3, mitochondrial
, tRNA modification GTPase GTPBP3, mitochondrial-like
, GTP-binding protein 3
, mitochondrial GTP-binding protein 1