Antibody Protein ELISA Kit from MyBioSource header Telephone 1.858.633.0165          
1.855.MyBioSource
Fax 1.858.633.0166    Email sales@mybiosource.com 
tel/fax
menu1 Home Products Ordering Company Help menu7
bottom
   MyBioSource rightarrow Antibody rightarrow FKRP  rightarrow LOG IN  rightarrow MY ACCOUNT  rightarrow CART CONTENTS  rightarrow CHECKOUT 
horiz bar
MyBioSource Menu separator
separator
ELISA Kit ELISA Kit
CLIA Kit CLIA Kit
PCR Kit PCR Kit
Monoclonal Antibody Monoclonal Antibody
Polyclonal Antibody Polyclonal Antibody
Secondary Antibody Secondary Antibody
Antigen Antigen
Biochemical Biochemical
cDNA Clone cDNA Clone
siRNA siRNA
Peptide Peptide
Recombinant/Purified Protein Rec./Purified Protein

Custom ELISA Kit Custom ELISA Kit
Custom Protein Custom Protein
Custom Antibody Custom Antibody
Antibody Matched Pairs Antibody Matched Pairs
Antibody & Corresponding Blocking Peptide Pairs Antibody Peptide Pairs
Phospho-Specific Antibodies Phospho Antibodies
Products by Disease Products by Disease
Products by Pathway Products by Pathway
Products by Tissue Products by Tissue

arrow Advanced Search
arrow Submit Technical Q&A
arrow International Distributors
arrow Contact Us
separator
Our Best Sellers moreseparator
separator
 • ST14 Antibody
 • BUB1 Antibody
 • Galectin 4 (GAL4) ELISA Kit
 • Suppressors of Cytokine Signaling 3 (SOCS3) ELISA Kit
 • Ss-B-La Antibody ELISA Kit
 • Coagulation Factor V (F5) ELISA Kit
 • Neurotrophin-1 (NT-1) ELISA Kit
 • Transferrin Receptor (TFR) ELISA Kit
 • Pro Brain Natriuretic Peptide (PROBNP) ELISA Kit
 • Bence-Jones Protein (BJP) ELISA Kit
 • Peroxisome Proliferators Activator beta (PPAR-beta) ELISA Kit
 • Forkhead Box Protein P3 (FOXP3) ELISA Kit
 • Platelet Associated Antibody IgM (PA-IgM) ELISA Kit
 • Bactericidal-Permeability Increasing Protein (BPI) ELISA Kit
 downarrow more ...
separator
separator
DatasheetFull DatasheetPrinter Friendly DatasheetPrint This DatasheetAdd to Compare ListHave Questions? Ask UsRequest for a Quotation today

anti-FKRP antibody :: Rabbit FKRP Antibody

Scan QR to view Datasheet Catalog #    MBS855737
Testing Data
Unit / Price
0.1 mg  /  $215 +1 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

FKRP, Antibody

 Also Known As   

FKRP Antibody

 Product Synonym Names    Fukutin-related protein; EC 2.-.-.-; FKRP
 Product Gene Name   

anti-FKRP antibody

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
Table BarTOPTable Bar
 OMIM    613153
 3D Structure    ModBase 3D Structure for Q9H9S5
Section Bar
 Host    Rabbit
 Species Reactivity    Human, Mouse, Rat
Section Bar
 Specificity    FKRP antibody detects endogenous levels of total FKRP protein.
 Purity/Purification    The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
 Form/Format    Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
Section Bar
 Immunogen    The antiserum was produced against synthesized peptide derived from N-terminal of human FKRP.
 Pathway    Transcription
Section Bar
 Preparation and Storage    Store at -20 degree C for 1 year
 Other Notes    Small volumes of anti-FKRP antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Table BarTOPTable Bar
 Product Categories/Family for anti-FKRP antibody    Cancer; Cardiovascular; Cell Biology; Epigenetics & Nuclear Signaling; Developmental Biologys; Immunology; Drug Discovery Products; Metabolism; Neuroscience; Signal Transduction; Stem Cells; Autophagy antibody
 Applications Tested/Suitable for anti-FKRP antibody   

Western Blot (WB), ELISA (EIA)

 Application Notes for anti-FKRP antibody    WB: 1:500~1:1000, ELISA: 1:20000
Section Bar
 Testing Data of anti-FKRP antibody    Western blot analysis of extracts from Jurkat cells and COLO205 cells, using FKRP antibody.
anti-FKRP antibody Testing Data image
Table BarTOPTable Bar
NCBI/Uniprot data below describe general gene information for FKRP. It may not necessarily be applicable to this product.
 NCBI GI #    89941475
 NCBI GeneID    79147
 NCBI Accession #    NP_001034974.1 [Other Products]
 NCBI GenBank Nucleotide #    NM_001039885.2 [Other Products]
Section Bar
 UniProt Primary Accession #    Q9H9S5 [Other Products]
 UniProt Secondary Accession #    A8K5G7 [Other Products]
 UniProt Related Accession #    Q9H9S5 [Other Products]
 Molecular Weight    54,568 Da
Table BarTOPTable Bar
 NCBI Official Full Name    fukutin-related protein
 NCBI Official Synonym Full Names    fukutin related protein
 NCBI Official Symbol    FKRP [Similar Products]
 NCBI Official Synonym Symbols   
MDC1C; LGMD2I; MDDGA5; MDDGB5; MDDGC5
[Similar Products]
 NCBI Protein Information    fukutin-related protein
 UniProt Protein Name    Fukutin-related protein
 Protein Family    Fukutin-related protein
 UniProt Gene Name    FKRP [Similar Products]
 UniProt Entry Name    FKRP_HUMAN
Section Bar
 NCBI Summary for FKRP    This gene encodes a protein which is targeted to the medial Golgi apparatus and is necessary for posttranslational modification of dystroglycan. Mutations in this gene have been associated with congenital muscular dystrophy, mental retardation, and cerebellar cysts. Several alternatively spliced transcript variants of this gene have been described, but the full-length nature of some of these variants has not been determined. [provided by RefSeq, Oct 2008]
Section Bar
 UniProt Comments for FKRP    FKRP: Could be a transferase involved in the modification of glycan moieties of alpha-dystroglycan (DAG1). Defects in FKRP are the cause of muscular dystrophy- dystroglycanopathy congenital with brain and eye anomalies type A5 (MDDGA5). MDDGA5 is an autosomal recessive disorder characterized by congenital muscular dystrophy associated with cobblestone lissencephaly and other brain anomalies, eye malformations, profound mental retardation, and death usually in the first years of life. Included diseases are the more severe Walker-Warburg syndrome and the slightly less severe muscle-eye- brain disease. Defects in FKRP are the cause of muscular dystrophy- dystroglycanopathy congenital with or without mental retardation type B5 (MDDGB5). MDDGB5 is a congenital muscular dystrophy characterized by a severe phenotype with inability to walk, muscle hypertrophy, marked elevation of serum creatine kinase, a secondary deficiency of laminin alpha2, and a marked reduction in alpha-dystroglycan expression. Only a subset of MDDGB5 patients have brain involvements. Defects in FKRP are the cause of muscular dystrophy- dystroglycanopathy limb-girdle type C5 (MDDGC5); also known as limb-girdle muscular dystrophy type 2I. MDDGC5 is an autosomal recessive disorder with age of onset ranging from childhood to adult life, and variable severity. Clinical features include proximal muscle weakness, waddling gait, calf hypertrophy, cardiomyopathy and respiratory insufficiency. A reduction of alpha-dystroglycan and laminin alpha-2 expression can be observed on skeletal muscle biopsy from MDDGC5 patients. Belongs to the LicD transferase family.

Protein type: Transferase; Membrane protein, integral; EC 2.-.-.-

Chromosomal Location of Human Ortholog: 19q13.32

Cellular Component: dystrophin-associated glycoprotein complex; Golgi membrane; Golgi apparatus; extracellular space; rough endoplasmic reticulum; integral to membrane; sarcolemma

Molecular Function: transferase activity

Biological Process: protein amino acid O-linked mannosylation; protein processing

Disease: Muscular Dystrophy-dystroglycanopathy (congenital With Or Without Mental Retardation), Type B, 5; Muscular Dystrophy-dystroglycanopathy (congenital With Brain And Eye Anomalies), Type A, 5; Muscular Dystrophy-dystroglycanopathy (limb-girdle), Type C, 5; Muscular Dystrophy-dystroglycanopathy (congenital With Brain And Eye Anomalies), Type A, 1
Table BarTOPTable Bar
 

Product References and Citations for anti-FKRP antibody

   Brockington M., Am. J. Hum. Genet. 69:1198-1209(2001).
Ota T., Nat. Genet. 36:40-45(2004).
Brockington M., Hum. Mol. Genet. 10:2851-2859(2001).
------------------------------------------------------------------------------------------------------------------------------------------
Section Bar
 Research Articles on FKRP    1. This study demonistrated that the higher frequency of LGMD2I with cardiomyopathy in mutation of FKRP in Taiwanese patients.
Table BarTOPTable Bar
 Precautions    All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
 Disclaimer    While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
Table BarTOPTable Bar
Products associated with anti-FKRP antibodyDiseases associated with anti-FKRP antibody
 Reference Product  PubMed Publications
 POMT1 antibody  >46 publications with FKRP and POMT1
 POMGNT1 antibody  >44 publications with FKRP and POMGNT1
 POMT2 antibody  >39 publications with FKRP and POMT2
 DAG1 antibody  >15 publications with FKRP and DAG1
 TRIM32 antibody  >5 publications with FKRP and TRIM32
 DMD antibody  >5 publications with FKRP and DMD
 LAMA2 antibody  >4 publications with FKRP and LAMA2
 SGCA antibody  >2 publications with FKRP and SGCA
 SGCG antibody  >1 publications with FKRP and SGCG
 Disease Name  Pubmed Publications
 Walker-Warburg Syndrome Antibodies  >47 publications with FKRP and Walker-Warburg Syndrome
 Eye Abnormalities Antibodies  >26 publications with FKRP and Eye Abnormalities
 Nervous System Malformations Antibodies  >17 publications with FKRP and Nervous System Malformations
 Hypertrophy Antibodies  >9 publications with FKRP and Hypertrophy
 Inflammation Antibodies  >4 publications with FKRP and Inflammation
 Fibrosis Antibodies  >3 publications with FKRP and Fibrosis
 Kidney Diseases Antibodies  >2 publications with FKRP and Kidney Diseases
 Neural Tube Defects Antibodies  >1 publications with FKRP and Neural Tube Defects
 Heart Defects, Congenital Antibodies  >1 publications with FKRP and Heart Defects, Congenital
 Learning Disorders Antibodies  >1 publications with FKRP and Learning Disorders
Organs/Tissues associated with anti-FKRP antibody
 Organ/Tissue Name  Pubmed Publications
 Muscle Antibodies  >110 publications with FKRP and Muscle
 Brain Antibodies  >66 publications with FKRP and Brain
 Eye Antibodies  >50 publications with FKRP and Eye
 Blood Antibodies  >13 publications with FKRP and Blood
 Heart Antibodies  >9 publications with FKRP and Heart
 Embryonic Tissue Antibodies  >6 publications with FKRP and Embryonic Tissue
 Nerve Antibodies  >6 publications with FKRP and Nerve
 Vascular Antibodies  >2 publications with FKRP and Vascular
 Connective Tissue Antibodies  >2 publications with FKRP and Connective Tissue
 Adipose Tissue Antibodies  >2 publications with FKRP and Adipose Tissue
Table BarTOPTable Bar
horiz bar
 SSL   Follow us on Facebook Follow us onTwitter Follow us on Google Plus Connect us on LinkedIn Subscribe to our RSS Feed for latest products and special promotions