Recombinant Anti-Fukutin antibody [EPR7913] (ab131280)
Key features and details
- Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply
- Rabbit monoclonal [EPR7913] to Fukutin
- Suitable for: WB, IHC-P
- Reacts with: Human
Related conjugates and formulations
Overview
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Product name
Anti-Fukutin antibody [EPR7913]
See all Fukutin primary antibodies -
Description
Rabbit monoclonal [EPR7913] to Fukutin -
Host species
Rabbit -
Tested applications
Suitable for: WB, IHC-Pmore details
Unsuitable for: Flow Cyt or IP -
Species reactivity
Reacts with: Human
Predicted to work with: Mouse, Rat -
Immunogen
Synthetic peptide. This information is proprietary to Abcam and/or its suppliers.
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Positive control
- HeLa, A549, fetal heart, and BxPC-3 lysates; Human kidney tissue.
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General notes
This product is a recombinant monoclonal antibody, which offers several advantages including:
- - High batch-to-batch consistency and reproducibility
- - Improved sensitivity and specificity
- - Long-term security of supply
- - Animal-free production
Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMAb® patents.
Properties
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Form
Liquid -
Storage instructions
Shipped 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 buffer
pH: 7.20
Preservative: 0.01% Sodium azide
Constituents: 9% PBS, 40% Glycerol (glycerin, glycerine), 0.05% BSA, 50% Tissue culture supernatant -
Concentration information loading...
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Purity
Protein A purified -
Clonality
Monoclonal -
Clone number
EPR7913 -
Isotype
IgG -
Research areas
Associated products
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Alternative Versions
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Isotype control
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Positive Controls
Applications
The Abpromise guarantee
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.
Application | Abreviews | Notes |
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WB |
1/1000 - 1/10000. Predicted molecular weight: 53 kDa.
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IHC-P |
1/100 - 1/250. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.
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Notes |
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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. |
Target
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Function
May 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 specificity
Widely 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 disease
Defects 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 similarities
Belongs to the licD transferase family. -
Cellular localization
Golgi apparatus membrane. - Information by UniProt
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Database links
- Entrez Gene: 2218 Human
- Entrez Gene: 246179 Mouse
- Entrez Gene: 362520 Rat
- Omim: 607440 Human
- SwissProt: O75072 Human
- SwissProt: Q8R507 Mouse
- Unigene: 55777 Human
- Unigene: 247210 Mouse
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Alternative names
- CMD1X antibody
- FCMD antibody
- FCMD gene antibody
see all
Images
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All lanes : Anti-Fukutin antibody [EPR7913] (ab131280) at 1/1000 dilution
Lane 1 : HeLa cell lysate
Lane 2 : A549 cell lysate
Lane 3 : Fetal heart tissue lysate
Lane 4 : BxPC-3 cell lysate
Lysates/proteins at 10 µg per lane.
Secondary
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.
Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.
Protocols
Datasheets and documents
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SDS download
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Datasheet download
References (4)
ab131280 has been referenced in 4 publications.
- Lesurf R et al. Whole genome sequencing delineates regulatory, copy number, and cryptic splice variants in early onset cardiomyopathy. NPJ Genom Med 7:18 (2022). PubMed: 35288587
- Pham TT et al. Characteristic expression of fukutin in gastric cancer among atomic bomb survivors. Oncol Lett 13:937-941 (2017). PubMed: 28356981
- Gerin I et al. ISPD produces CDP-ribitol used by FKTN and FKRP to transfer ribitol phosphate onto a-dystroglycan. Nat Commun 7:11534 (2016). PubMed: 27194101
- Oo HZ et al. Fukutin, identified by the Escherichia coli ampicillin secretion trap (CAST) method, participates in tumor progression in gastric cancer. Gastric Cancer 19:443-452 (2016). PubMed: 26223471