FARS2 Antibody
-
中文名稱：FARS2兔多克隆抗體
-
貨號：CSB-PA008429GA01HU
-
規格：￥3,900
-
其他：
產品詳情
-
Uniprot No.：
-
基因名：FARS2
-
別名：dJ236A3.1 (phenylalanine tRNA synthetase) antibody; dJ520B18.2 (FARS1 (phenylalanine tRNA synthetase)) antibody; FARS1 antibody; Fars2 antibody; HSPC320 antibody; Phenylalanine translase antibody; Phenylalanine tRNA ligase 2; mitochondrial antibody; Phenylalanine tRNA ligase antibody; Phenylalanine tRNA synthetase 1 (mitochondrial) antibody; Phenylalanine tRNA synthetase 2 (mitochondrial) antibody; Phenylalanine--tRNA ligase antibody; Phenylalanyl tRNA synthetase 2 antibody; Phenylalanyl-tRNA synthetase; mitochondrial antibody; PheRS antibody; SYFM_HUMAN antibody
-
宿主：Rabbit
-
反應種屬：Human,Mouse,Rat
-
免疫原：Human FARS2
-
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
-
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：Is responsible for the charging of tRNA(Phe) with phenylalanine in mitochondrial translation. To a lesser extent, also catalyzes direct attachment of m-Tyr (an oxidized version of Phe) to tRNA(Phe), thereby opening the way for delivery of the misacylated tRNA to the ribosome and incorporation of ROS-damaged amino acid into proteins.
-
基因功能參考文獻：
- Based on the phenotypic data of previously reported subjects and the two subjects reported here, we conclude that FARS2 deficiency can be associated with two phenotypes: (i) an epileptic phenotype, and (ii) a spastic paraplegia phenotype. PMID: 29126765
- in patients with drug-resistant infantile spasm syndrome, associated with focal seizures, mild metabolic changes, and cerebral atrophy with volume loss of white matter on MRI, mutations in FARS2 should be considered. PMID: 27549011
- Kinetic and structural changes in HsmtPheRS, induced by pathogenic mutations in human FARS2 have been described. PMID: 28419689
- A newly identified missense mutation in FARS2 causes autosomal-recessive spastic paraplegia. PMID: 26553276
- this study expands the phenotypic spectrum of FARS2 related disease and emphasizes intragenic deletion in the list of causative mutations. PMID: 25851414
- the three FARS2 mutations directly impair aminoacylation function and stability of mtPheRS, leading to a decrease in overall tRNA charging capacity. PMID: 22833457
- Two phenylalanyl-tRNA synthetase variants Ser57Cys and Asp280Ser both display wild-type aminoacylation activity and stability with respect to their free energies of unfolding, but are less stable at low hydrogen-ion concentration (pH). PMID: 21601574
- The recombinant human enzyme has been purified to homogeneity and crystallized in complex with phenylalanine and ATP. PMID: 17768348
- Formation of the PheRS-tRNAPhe complex in human mitochondria must be accompanied by considerable rearrangement of the anticodon binding domain upon tRNA binding. PMID: 18611382
- Mitochondrial and cytoplasmic phenylalanyl-tRNA synthetases (HsmtPheRS and HsctPheRS, respectively) catalyze direct attachment of m-Tyr to tRNA(Phe). PMID: 19549855
- these results indicate that conformational flexibility of the two functional modules in mtPheRS is essential for its phenylalanylation activity. PMID: 19737557
顯示更多
收起更多
-
相關疾病：Combined oxidative phosphorylation deficiency 14 (COXPD14); Spastic paraplegia 77, autosomal recessive (SPG77)
-
亞細胞定位：Mitochondrion matrix. Mitochondrion.
-
蛋白家族：Class-II aminoacyl-tRNA synthetase family
-
數據庫鏈接：
Most popular with customers
-
YWHAB Recombinant Monoclonal Antibody
Applications: ELISA, WB, IHC, IF, FC
Species Reactivity: Human, Mouse, Rat
-
Phospho-YAP1 (S127) Recombinant Monoclonal Antibody
Applications: ELISA, WB, IHC
Species Reactivity: Human
-
-
-
-
-
-
