Rabbit polyclonal FKRP antibody (A10194)

$69.00$259.00

Reactivity: Human
Applications: WB
Conjugation: Unconjugated
Supplier: ABclonal Inc.

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Rabbit polyclonal FKRP antibody (A10194)

Supplier: ABclonal Inc.

Recommended applications: WB

Recommended dilution:

WB 1:1000 – 1:3000

Recommended protocols: check protocols

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Alternative names:

Check alternative names for the antibody

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Fkrp antibody|LGMD2I antibody|MDC1C antibody|MGC2991 antibody|Anti-FKRP antibody (ab65243)

SCBT cat No: sc-48503|sc-374642|sc-48506|

Name

Rabbit polyclonal FKRP antibody

Catalogue No.

A10194

Reactivity

Human

Immunogen

Recombinant protein of human FKRP

Host

Rabbit

Applications

WB

Recommended dilution

WB 1:1000 – 1:3000

Clonality

Polyclonal

Conjugation

Unconjugated

Isotype

IgG

Molecular weight

Predicted: 55kDa/Observed: Refer to figures

Formulation

FKRP antibody was tube-contained.
Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Purification

FKRP antibody was purified using affinity purification.

Storage

Store at -20 Celsius degree. Avoid freeze / thaw cycles.

Database links

Human UniProt/Swiss-Prot:Q9H9S5

Protein function

Transferase involved in the biosynthesis of the phosphorylated O-mannosyl trisaccharide (N-acetylgalactosamine-beta-3-N-acetylglucosamine-beta-4-(phosphate-6-)mannose), a carbohydrate structure present in alpha-dystroglycan (DAG1), which is required for binding laminin G-like domain-containing extracellular proteins with high affinity.

Protein tissue specificity

Expressed predominantly in skeletal muscle, placenta, and heart and relatively weakly in brain, lung, liver kidney and pancreas.

Protein sequence and domain

Belongs to the LicD transferase family.

Protein post-translational modifications

N-glycosylated.

Protein cellular localization

Golgi apparatus membrane ; Single-pass type II membrane protein. Secreted. Cell membrane, sarcolemma . Rough endoplasmic reticulum. Note: According to some studies the N-terminal hydrophobic domain is cleaved after translocation to the Golgi apparatus and the protein is secreted. Localization at the cell membrane may require the presence of dystroglycan. At the Golgi apparatus localizes to the middle-to-trans-cisternae, as assessed by MG160 colocalization. Detected in rough endoplasmic reticulum in myocytes. In general, mutants associated with severe clinical phenotypes are retained within the endoplasmic reticulum.

Background

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]

Research area

All research areas>Transcription Regulators>FKRP
(View all antibody categories related to Transcription Regulators)

Note

AntibodyPlus can customize FKRP antibody according to your requirement, including bulk product size,etc. Please contact info@antibodyplus.com. AntibodyPlus provides antibody trial sample for your own antibody validation.

Product type

Primary antibody

Supplier

ABclonal Inc.

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