Product nameAnti-Fukutin antibody [EPR7913]
See all Fukutin primary antibodies
DescriptionRabbit monoclonal [EPR7913] to Fukutin
Tested applicationsSuitable for: ICC/IF, WB, IHC-Pmore details
Unsuitable for: Flow Cyt or IP
Species reactivityReacts with: Mouse, Rat, Human
Synthetic peptide within Human Fukutin aa 400-500. The exact sequence is proprietary.
- HeLa, A549, fetal heart, and BxPC-3 lysates; Human kidney tissue.
Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMab® patents
This product is a recombinant rabbit monoclonal antibody.
Storage instructionsShipped at 4°C. Store at -20°C. Stable for 12 months at -20°C.
Dissociation constant (KD)KD = 5.00 x 10 -11 M Learn more about KD
Storage bufferpH: 7.20
Preservative: 0.01% Sodium azide
Constituents: 9% PBS, 40% Glycerol, 0.05% BSA, 50% Tissue culture supernatant
PurityTissue culture supernatant
Our Abpromise guarantee covers the use of ab131280 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|ICC/IF||1/250 - 1/500.|
|WB||1/1000 - 1/10000. Predicted molecular weight: 53 kDa.|
|IHC-P||1/100 - 1/250. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.|
FunctionMay be a glycosyltransferase which participates in glycosylation of alpha-dystroglycan/DAG1. May interact with and reinforce a large complex encompassing the outside and inside of muscle membranes. Could be involved in brain development.
Tissue specificityWidely expressed with highest expression in brain, heart, pancreas and skeletal muscle. Expressed at similar levels in control fetal and adult brain, but is much reduced in Fukuyama-type congenital dystrophy (FCMD) brains. Expressed in migrating neurons, including Cajar-Retzius cells and adult cortical neurons, as well as hippocampal pyramidal cells and cerebellar Purkinje cells. No expression observed in the glia limitans, the subpial astrocytes (which contribute to basement membrane formation) or other glial cells. In the FCMD brain, neurons in regions with no dysplasia show fair expression, whereas transcripts are nearly undetectable in the overmigrated dysplastic region.
Involvement in diseaseDefects in FKTN are the cause of muscular dystrophy-dystroglycanopathy congenital with brain and eye anomalies type A4 (MDDGA4) [MIM:253800]; also called congenital muscular dystrophy Fukuyama type (FCMD) or Walker-Warburg syndrome FKTN-related. MDDGA4 is an autosomal recessive disorder characterized by congenital muscular dystrophy associated with cobblestone lissencephaly and other brain anomalies. Patients suffer from generalized skeletal muscle weakness and hypotonia from early infancy, mental retardation and seizures. Occasional features include optic atrophy, retinal detachment, cardiomyopathy.
Defects in FKTN are the cause of muscular dystrophy-dystroglycanopathy congenital without mental retardation type B4 (MDDGB4) [MIM:613152]. An autosomal recessive disorder characterized by congenital muscular dystrophy and evidence of dystroglycanopathy. Features included increased serum creatine kinase, generalized weakness, mild white matter changes on brain MRI in some cases, and absence of mental retardation.
Defects in FKTN are the cause of muscular dystrophy-dystroglycanopathy limb-girdle type C4 (MDDGC4) [MIM:611588]. MDDGC4 is an autosomal recessive degenerative myopathy characterized by progressive weakness of the pelvic and shoulder girdle muscles and elevated serum creatine kinase. The severity of the disease depends on age at onset which may vary from early to late childhood or even adulthood. MDDGC4 is a novel form of LGMD2 and has no brain involvement and a remarkable clinical response to corticosteroids.
Defects in FKTN are the cause of cardiomyopathy dilated type 1X (CMD1X) [MIM:611615]; also called dilated cardiomyopathy with mild or no proximal muscle weakness. Dilated cardiomyopathy is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.
Sequence similaritiesBelongs to the licD transferase family.
Cellular localizationGolgi apparatus membrane.
- Information by UniProt
- CMD1X antibody
- FCMD antibody
- FCMD gene antibody
All lanes : Anti-Fukutin antibody [EPR7913] (ab131280) at 1/1000 dilution
Lane 1 : HeLa lysate
Lane 2 : A549 lysate
Lane 3 : Fetal heart lysate
Lane 4 : BxPC-3 lysate
Lysates/proteins at 10 µg per lane.
All lanes : HRP labelled goat anti-rabbit at 1/2000 dilution
Predicted band size: 53 kDa
Immunohistochemical analysis of paraffin-embedded Human kidney tissue labelling Fukutin with ab131280 at 1/100 dilution.
Equilibrium disassociation constant (KD)
Learn more about KD
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