Products for Research Use Only
RegisterLogin

FKRP rabbit pAb

CAT: 0855-ES7567-01Size: 50 µLDry Ice: NoHazardous: No
Product image 1
1 / 1
CAT#:0855-ES7567-01Size:50 µL
Selected
24/48H Stock Items & 2 to 6 Weeks non Stock Items.
Quick Request Actions
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]
Description
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],
UniProt
Q9H9S5
Swiss Prot
Q9H9S5
Reactivity
Human; Mouse; Rat
Immunogen
The antiserum was produced against synthesized peptide derived from human FKRP. AA range:1-50
Clonality
Polyclonal
Source
Rabbit
Applications
WB; IF; ELISA
Concentration
1 mg/ml
Dilution
Western Blot: 1/500 - 1/2000. Immunofluorescence: 1/200 - 1/1000. ELISA: 1/20000. Not yet tested in other applications.
Molecular Weight
50kD
Storage Conditions
-20°C/1 year
Observed Molecular Weight
50kD
Fragment
IgG
Subcellular Location
Golgi apparatus membrane ; Single-pass type II membrane protein . Secreted . Cell membrane, sarcolemma . Rough endoplasmic reticulum . Cytoplasm . According to some studies the N-terminal hydrophobic domain is cleaved after translocation to the Golgi apparatus and the protein is secreted (PubMed:19900540) . Localization at the cell membrane may require the presence of dystroglycan (By similarity) . At the Golgi apparatus localizes to the middle-to-trans-cisternae, as assessed by MG160 colocalization. Detected in rough endoplasmic reticulum in myocytes (PubMed:17554798, PubMed:21886772) . In general, mutants associated with severe clinical phenotypes are retained within the endoplasmic reticulum (PubMed:15213246) . .
Other Product Names
FKRP; Fukutin-related protein
Gene ID (Human)
79147

Related Products

CatalogName
ES4666-01RAB3IP rabbit pAb
ES20751-01Gab1 Rabbit rabbit pAb
ES20703-01JAK2 Rabbit rabbit pAb
ES20758-01ERK1 Rabbit rabbit pAb
ES20830-01ATG14L Rabbit rabbit pAb
ES20829-01ATG4a Rabbit rabbit pAb
ES20658-01NM23A Rabbit rabbit pAb
ES20826-01ATG5 Rabbit rabbit pAb
ES20659-01NBR1 Rabbit rabbit pAb
ES20825-01ATG7 Rabbit rabbit pAb

Alternative Products

CatalogName
A10194-01FKRP Rabbit pAb
GTR17870273Fkrp Rabbit Polyclonal Antibody
NBP3-04945-100ulRabbit Polyclonal FKRP Antibody
GB111929Anti-FKRP Rabbit pAb
APR17814N-01FKRP Rabbit pAb (APR17814N)
YLD3123-01Rabbit anti-FKRP Antibody
316133FKRP

Popular Products