产品名称 Anti-GFPT1抗体[EPR4854] 参阅全部 GFPT1 一抗
兔单克隆抗体[EPR4854] to GFPT1
WB, IP, IHC-P more details 不适用于:
Flow Cyt or ICC
Mouse, Rat, Human
Synthetic peptide corresponding to residues in Human GFPT1.
Human testis tissue; Human placenta lysate, 293T, JAR and HeLa cell line lysates.
This product is a recombinant rabbit monoclonal antibody.
Produced using Abcam’s RabMAb
® technology. RabMAb ® technology is covered by the following U.S. Patents, No. 5,675,063 and/or 7,429,487.
存放说明 Shipped at 4°C. Store at -20°C. Stable for 12 months at -20°C.
存储溶液 PBS 49%,Sodium azide 0.01%,Glycerol 50%,BSA 0.05%
纯度 Tissue culture supernatant
Abpromise guarantee covers the use of
in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
1/1000 - 1/10000. Detects a band of approximately 79 kDa (predicted molecular weight: 79 kDa).
1/10 - 1/100.
1/100 - 1/250. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.
应用说明 Is unsuitable for Flow Cyt or ICC.
功能 Controls the flux of glucose into the hexosamine pathway. Most likely involved in regulating the availability of precursors for N- and O-linked glycosylation of proteins.
组织特异性 Isoform 1 is predominantly expressed in skeletal muscle. Not expressed in brain. Seems to be selectively expressed in striated muscle.
通路 Nucleotide-sugar biosynthesis; UDP-N-acetyl-alpha-D-glucosamine biosynthesis; alpha-D-glucosamine 6-phosphate from D-fructose 6-phosphate: step 1/1.
疾病相关 Defects in GFPT1 are the cause of limb-girdle myasthenia with tubular aggregates (LGMTA) [MIM:610542]. A congenital myasthenic syndrome characterized by onset of proximal muscle weakness in the first decade. Individuals with this condition have a recognizable pattern of weakness of shoulder and pelvic girdle muscles, and sparing of ocular or facial muscles. EMG classically shows a decremental response to repeated nerve stimulation, a sign of neuromuscular junction dysfunction. Affected individuals show a favorable response to acetylcholinesterase (AChE) inhibitors.
序列相似性 Contains 1 glutamine amidotransferase type-2 domain. Contains 2 SIS domains.
Information by UniProt
D-fructose-6-phosphate amidotransferase 1 antibody
Anti-GFPT1 antibody [EPR4854] 图像
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-GFPT1 antibody [EPR4854] (ab125069)
ab125069, at a 1/100 dilution, staining GFPT1 in paraffin-embedded Human testis tissue by immunohistochemistry.
Western blot - Anti-GFPT1 antibody [EPR4854] (ab125069)
All lanes : Anti-GFPT1 antibody [EPR4854] (ab125069) at 1/1000 dilution Lane 1 : 293T cell lysate Lane 2 : JAR cell lysate Lane 3 : HeLa cell lysate Lane 4 : Human placenta lysate Lysates/proteins at 10 µg per lane. Secondary Goat anti-Rabbit HRP at 1/2000 dilution Developed using the ECL technique Predicted band size : 79 kDa
Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"