GTPBP3 Antibody
-
中文名稱：GTPBP3兔多克隆抗體
-
貨號：CSB-PA010034GA01HU
-
規格：￥3,900
-
其他：
產品詳情
-
Uniprot No.：
-
基因名：GTPBP3
-
別名：GTPBP3 antibody; MTGP1 antibody; tRNA modification GTPase GTPBP3 antibody; mitochondrial antibody; GTP-binding protein 3 antibody; Mitochondrial GTP-binding protein 1 antibody
-
宿主：Rabbit
-
反應種屬：Human,Mouse,Rat
-
免疫原：Human GTPBP3
-
免疫原種屬：Homo sapiens (Human)
-
抗體亞型：IgG
-
純化方式：Antigen Affinity Purified
-
濃度：It differs from different batches. Please contact us to confirm it.
-
保存緩沖液：PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
-
產品提供形式：Liquid
-
應用范圍：ELISA,WB,IHC
-
Protocols：
-
儲存條件：Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
-
貨期：Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
-
用途：For Research Use Only. Not for use in diagnostic or therapeutic procedures.
產品評價
相關產品
靶點詳情
-
功能：GTPase involved in the 5-carboxymethylaminomethyl modification (mnm(5)s(2)U34) of the wobble uridine base in mitochondrial tRNAs.
-
基因功能參考文獻：
- Defects in the mitochondrial tRNA modification enzymes MTO1 and GTPBP3 promote different metabolic reprogramming through a HIF-PPARgamma-UCP2-AMPK axis. PMID: 29348686
- 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. PMID: 26642043
- Most individuals with GTPBP3 mutations developed neurological symptoms and MRI involvement of thalamus, putamen, and brainstem resembling Leigh syndrome. PMID: 25434004
- GTPBP3 localizes in the mitochondria and is a deafness-associated homolog of yeast MSS1. PMID: 12370316
- Phenotype of non-syndromic deafness associated with the mitochondrial A1555G mutation is modulated by mitochondrial RNA modifying enzymes MTO1 and GTPBP3. PMID: 15542390
- 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. PMID: 18852288
顯示更多
收起更多
-
相關疾病：Combined oxidative phosphorylation deficiency 23 (COXPD23)
-
亞細胞定位：Mitochondrion.
-
蛋白家族：TRAFAC class TrmE-Era-EngA-EngB-Septin-like GTPase superfamily, TrmE GTPase family
-
組織特異性：Ubiquitously expressed.
-
數據庫鏈接：
Most popular with customers
-
-
Phospho-YAP1 (S127) Recombinant Monoclonal Antibody
Applications: ELISA, WB, IHC
Species Reactivity: Human
-
-
-
-
-
-
