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Rabbit monoclonal [EPR4854] to GFPT1
IHC-P more details Unsuitable for:
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.
® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMab ® patents
Shipped at 4°C. Store at -20°C. Stable for 12 months at -20°C.
PBS 49%,Sodium azide 0.01%,Glycerol 50%,BSA 0.05%
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.
Is unsuitable for Flow Cyt or ICC.
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.
Isoform 1 is predominantly expressed in skeletal muscle. Not expressed in brain. Seems to be selectively expressed in striated muscle.
Nucleotide-sugar biosynthesis; UDP-N-acetyl-alpha-D-glucosamine biosynthesis; alpha-D-glucosamine 6-phosphate from D-fructose 6-phosphate: step 1/1.
Involvement in disease
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.
Contains 1 glutamine amidotransferase type-2 domain.
Contains 2 SIS domains.
Information by UniProt
D-fructose-6-phosphate amidotransferase 1 antibody
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"