GTP Binding Protein 3, Mitochondrial (GTPBP3)

[Edit]

MSS1; GTPBG3; MTGP1; THDF1; Mitochondrial GTP-binding protein 1; tRNA modification GTPase GTPBP3, mitochondrial

GTP Binding Protein 3, Mitochondrial (GTPBP3)
Mss1 is a yeast mitochondrial GTP-binding protein that functionally interacts with 15S rRNA. The variants are generated by alternative splicing and through the use of alternate polyadenylation signals. Two variants differ only in their 3-prime UTRs and encode an identical 492-amino acid protein with a calculated molecular mass of 52.0 kD. Two other variants encode an identical 471-amino acid protein, and another variant encodes a 524-amino acid protein. GTPBP3 contains a typical mitochondria targeting presequence, 2 GTP-binding domains, a p21 RAS-like domain, and an SRC homology-3 (SH3) domain. GTPBP3 shows extensive conservation of amino acid sequences with Mss1 homologs from several diverse species, including bacteria, yeast, and nematode. Mouse and human GTPBP3 share 81% amino acid identity.

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant GTP Binding Protein 3, Mitochondrial (GTPBP3) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to GTP Binding Protein 3, Mitochondrial (GTPBP3) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to GTP Binding Protein 3, Mitochondrial (GTPBP3) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for GTP Binding Protein 3, Mitochondrial (GTPBP3) CLIA Kit Customized Service Offer
n/a ELISA Kit for GTP Binding Protein 3, Mitochondrial (GTPBP3) ELISA Kit Customized Service Offer

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant GTP Binding Protein 3, Mitochondrial (GTPBP3) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to GTP Binding Protein 3, Mitochondrial (GTPBP3) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to GTP Binding Protein 3, Mitochondrial (GTPBP3) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for GTP Binding Protein 3, Mitochondrial (GTPBP3) CLIA Kit Customized Service Offer
n/a ELISA Kit for GTP Binding Protein 3, Mitochondrial (GTPBP3) ELISA Kit Customized Service Offer

Organism species: Rattus norvegicus (Rat)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant GTP Binding Protein 3, Mitochondrial (GTPBP3) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to GTP Binding Protein 3, Mitochondrial (GTPBP3) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to GTP Binding Protein 3, Mitochondrial (GTPBP3) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for GTP Binding Protein 3, Mitochondrial (GTPBP3) CLIA Kit Customized Service Offer
n/a ELISA Kit for GTP Binding Protein 3, Mitochondrial (GTPBP3) ELISA Kit Customized Service Offer
  1. "A human mitochondrial GTP binding protein related to tRNA modification may modulate phenotypic expression of the deafness-associated mitochondrial 12S rRNA mutation."Mol. Cell. Biol. 22:7701-7711(2002) [PubMed] [Europe PMC] [Abstract]
  2. "Complete sequencing and characterization of 21,243 full-length human cDNAs." Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
  3. "The DNA sequence and biology of human chromosome 19." Nature 428:529-535(2004) [PubMed] [Europe PMC] [Abstract]
  4. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
  5. "Phenotype of non-syndromic deafness associated with the mitochondrial A1555G mutation is modulated by mitochondrial RNA modifying enzymes MTO1 and GTPBP3."Mol. Genet. Metab. 83:199-206(2004) [PubMed] [Europe PMC] [Abstract]
  6. "Mutations in GTPBP3 cause a mitochondrial translation defect associated with hypertrophic cardiomyopathy, lactic acidosis, and encephalopathy." Am. J. Hum. Genet. 95:708-720(2014) [PubMed] [Europe PMC] [Abstract]