GTP Binding Protein 3 (Mitochondrial) Proteins (GTPBP3)

This locus encodes a GTP-binding protein. Additionally we are shipping GTPBP3 Antibodies (37) and GTPBP3 Kits (6) and many more products for this protein.

list all proteins Gene Name GeneID UniProt
Rat GTPBP3 GTPBP3 290633 Q5PQQ1
GTPBP3 70359 Q923K4
GTPBP3 84705 Q969Y2
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Top GTPBP3 Proteins at

Showing 5 out of 5 products:

Catalog No. Origin Source Conjugate Images Quantity Supplier Delivery Price Details
Insect Cells Human His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 50 Days
Insect Cells Mouse His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 50 Days
Wheat germ Human GST tag 10 μg Log in to see 11 to 12 Days
HEK-293 Cells Human Myc-DYKDDDDK Tag Validation with Western Blot 20 μg Log in to see 11 Days
Yeast Zebrafish His tag   1 mg Log in to see 60 to 71 Days

GTPBP3 Proteins by Origin and Source

Origin Expressed in Conjugate
Mouse (Murine)

Human , ,
, ,

More Proteins for GTP Binding Protein 3 (Mitochondrial) (GTPBP3) Interaction Partners

Zebrafish GTP Binding Protein 3 (Mitochondrial) (GTPBP3) interaction partners

  1. defective expression of gtpbp3 related to tRNA modification alters the mitochondrial function and development of zebrafish

Mouse (Murine) GTP Binding Protein 3 (Mitochondrial) (GTPBP3) interaction partners

  1. These observations suggest that the mouse Gtpbp3 is an evolutionarily conserved mitochondrial GTP-binding protein involved in the tRNA modification.

Human GTP Binding Protein 3 (Mitochondrial) (GTPBP3) interaction partners

  1. Low GTPBP3 expression is associated with Mitochondrial diseases in neoplasms.

  2. Defects in the mitochondrial tRNA modification enzymes MTO1 and GTPBP3 promote different metabolic reprogramming through a HIF-PPARgamma-UCP2-AMPK axis.

  3. GTPBP3 defective expression is associated with an mitochondrial-tRNA hypomodification status.GTPBP3 plays a role in the regulation of UCP2 and MCP1 proteins through AMPK signaling.

  4. Results show that post-transcriptional expression of GTPBP3, MTO1 and TRMU genes is down-regulated, leading to mt-tRNA hypomodification and contributing to mitochondrial dysfunction in MELAS cybrids.

  5. Most individuals with GTPBP3 mutations developed neurological symptoms and MRI involvement of thalamus, putamen, and brainstem resembling Leigh syndrome.

  6. GTPBP3 localizes in the mitochondria and is a deafness-associated homolog of yeast MSS1.

  7. Phenotype of non-syndromic deafness associated with the mitochondrial A1555G mutation is modulated by mitochondrial RNA modifying enzymes MTO1 and GTPBP3.

  8. Data show that the two most abundant GTPBP3 isoforms exhibit moderate affinity for guanine nucleotides like their bacterial homologue, MnmE, although they hydrolyze GTP at a 100-fold lower rate.

GTPBP3 Protein Profile

Protein Summary

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.

Gene names and symbols associated with GTPBP3

  • GTP binding protein 3, mitochondrial (GTPBP3)
  • GTP binding protein 3 (Gtpbp3)
  • GTP binding protein 3, mitochondrial (gtpbp3)
  • 2410009F13Rik protein
  • AI607903 protein
  • GTPBG3 protein
  • GTPBP3 protein
  • MSS1 protein
  • MTGP1 protein
  • THDF1 protein
  • zgc:112394 protein

Protein level used designations for GTPBP3

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

484838 Canis lupus familiaris
539656 Bos taurus
100070158 Equus caballus
100392904 Callithrix jacchus
100474937 Ailuropoda melanoleuca
290633 Rattus norvegicus
553807 Danio rerio
70359 Mus musculus
84705 Homo sapiens
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