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anti-Fukutin Related Protein (FKRP) Antibodies

FKRP encodes a protein which is targeted to the medial Golgi apparatus and is necessary for posttranslational modification of dystroglycan. Additionally we are shipping Fukutin Related Protein Proteins (6) and Fukutin Related Protein Kits (2) and many more products for this protein.

list all antibodies Gene Name GeneID UniProt
FKRP 79147 Q9H9S5
FKRP 243853 Q8CG64
FKRP 308390  
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Top anti-Fukutin Related Protein Antibodies at antibodies-online.com

Showing 10 out of 49 products:

Catalog No. Reactivity Host Conjugate Application Images Quantity Supplier Delivery Price Details
Human Rabbit Un-conjugated WB WB Suggested Anti-Fkrp Antibody Titration: 0.2-1 ug/mlELISA Titer: 1:62500Positive Control: Mouse Heart WB Suggested Anti-Fkrp  Antibody Titration: 0.2-1 µg/mL ELISA Titer: 1:.2500  Positive Control: Mouse Heart 100 μL Log in to see 2 to 3 Days
$289.00
Details
Human Rabbit Un-conjugated ELISA, WB Western blot analysis of extracts from Jurkat/COLO205 cells, using FKRP Antibody. The lane on the right is treated with the synthesized peptide. 100 μg Log in to see 2 to 3 Days
$302.50
Details
Human Mouse Un-conjugated WB FKRP Antibody (C-term)  western blot analysis in HL-60 cell line lysates (35ug/lane).This demonstrates the FKRP antibody detected the FKRP protein (arrow). 0.1 mL Log in to see 10 to 11 Days
$324.50
Details
Human Rabbit Un-conjugated WB FKRP Antibody (Center) (ABIN658206) western blot analysis in HL-60 cell line lysates (35 µg/lane). This demonstrates the FKRP antibody detected the FKRP protein (arrow). 400 μL Log in to see 10 to 11 Days
$324.50
Details
Human Mouse Un-conjugated WB FKRP Antibody (C-term)  western blot analysis in HL-60 cell line lysates (35ug/lane).This demonstrates the FKRP antibody detected the FKRP protein (arrow). 400 μL Log in to see 10 to 11 Days
$324.50
Details
Human Rabbit Un-conjugated ELISA, WB 100 μL Log in to see 8 to 10 Days
$478.50
Details
Mouse Rabbit Un-conjugated WB 50 μg Log in to see 8 to 10 Days
$551.83
Details
Human Rabbit Un-conjugated ELISA, WB 100 μg Log in to see 11 to 16 Days
$412.86
Details
Human Mouse Un-conjugated IHC (p), WB Human Liver: Formalin-Fixed, Paraffin-Embedded (FFPE) 50 μL Log in to see 8 to 10 Days
$451.00
Details
Human Rabbit Un-conjugated WB Mouse Liver; WB Suggested Anti-Fkrp Antibody. . Titration: 1.0 ug/ml. . Positive Control: Mouse liver; Fkrp antibody - C-terminal region in Mouse Liver cells using Western Blot 50 μg Log in to see 6 to 8 Days
$401.50
Details

FKRP Antibodies by Reactivity, Application, Clonality and Conjugate

Attributes Applications Host Clonality Conjugate
Human ,
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Mouse (Murine)


Rat (Rattus)


More Antibodies against Fukutin Related Protein Interaction Partners

Zebrafish Fukutin Related Protein (FKRP) interaction partners

  1. Fukutin (show FKTN Antibodies) and FKRP have functions that affect ocular development in zebrafish independently of dystroglycan (show DAG1 Antibodies).

  2. Muscle pathology in embryos lacking Fukutin (show FKTN Antibodies) or FKRP is different from loss of dystroglycan (show DAG1 Antibodies); knockdown of Fukutin (show FKTN Antibodies) or FKRP leads to a notochord defect and a perturbation of laminin expression before muscle degeneration.

  3. Zebrafish are a useful animal model to reveal the mechanism of muscular dystrophiescaused by mutations in FKRP gene.

  4. It was found that downregulating FKRP in the zebrafish results in embryos which develop a range of abnormalities reminiscent of the developmental defects observed in human muscular dystrophies associated with mutations in FKRP

Human Fukutin Related Protein (FKRP) interaction partners

  1. This study demonistrated that the higher frequency of LGMD2I with cardiomyopathy in mutation of FKRP in Taiwanese patients.

  2. FKRP co-localises with the middle-to-trans-Golgi marker MG160 (show GLG1 Antibodies), between the myofibrils in human rectus femoris muscle fibres.

  3. Mutations in FKRP lead to a glycosylation defect and subsequently downregulation of alpha-dystroglycan which constitutes an essential component of the proteoglycan (show Vcan Antibodies)-dystrophin (show DMD Antibodies) complex.

  4. Study revealed a large homozygous block at the LGMD2I locus, and direct sequencing of FKRP encoding fukutin-related-protein detected the common homozygous c.826 C>A (p.Leu276Ile) mutation.

  5. Two novel heterozygous mutations (c.208T>A and c.1030G>T) in the FKRP gene were identified in Chinese brothers with progressive shoulder and pelvic muscle weakness

  6. This study identified FKRP mutations on both alleles in 88 patients from 69 families with Limb Girdle Muscular Dystrophy Type 2I.

  7. two siblings carrying a homozygous mutation in the start codon of FKRP that is likely to result in a loss of functional FKRP protein. The clinical phenotype of the patients was consistent with Walker-Warburg syndrome

  8. our study confirms that typical clinical symptoms (calf hypertrophy, cardiac involvement, mild LGMD) of LGMD2I due the homozygous c.826C[A mutation, are rather frequent in Germany

  9. Co-injection of fish or human FKRP mRNA along with the morpholino restored normal development and alpha-dystroglycan glycosylation.

  10. Alteration of the secretion pathway by different mutations may contribute to wide variations in phenotypes associated with FKRP-related diseases such as muscular dystrophy.

Mouse (Murine) Fukutin Related Protein (FKRP) interaction partners

  1. Reduced Fkrp levels in skeletal muscle are associated with a progressive muscular dystrophy

  2. FKRP mutations are associated with muscular dystrophy.

  3. a reduction in Fkrp influences the ability of tissue-specific forms of alpha-dystroglycan to direct the deposition of several laminin isoforms

  4. investigation of the functional roles of FKRP in muscular dystrophies; FKRP is essential for the functional glycosylation of alpha-dystroglycan;both the mutation itself and the levels of FKRP expression are equally critical for the survival of the animals

  5. Basement membrane (BM) fragility may underlie abnormal phenotypes in fukutin (show FKTN Antibodies)-null embryos, and maintenance of BM function may require fukutin (show FKTN Antibodies)-mediated glycosylation of alpha-dystroglycan early in embryonic development.

  6. data offer the first evidence of a fukutin-related protein (FKRP) complex in muscle and suggest that FKRP may influence the glycosylation status of dystroglycan (show DAG1 Antibodies) from within the sarcolemmal dystrophin (show DMD Antibodies)-glycoprotein complex

  7. results suggest the generation of a mouse model for FKRP related muscular dystrophy requires a knock-down rather than a knock-in strategy in order to give rise to a disease phenotype.

Fukutin Related Protein (FKRP) Antigen Profile

Protein Summary

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.

Gene names and symbols associated with FKRP

  • fukutin related protein (FKRP) antibody
  • fukutin related protein (fkrp) antibody
  • fukutin related protein (Fkrp) antibody
  • A830029B19Rik antibody
  • AI842067 antibody
  • AI847300 antibody
  • LGMD1I antibody
  • LGMD2I antibody
  • MDC1C antibody
  • MDDGA5 antibody
  • MDDGB5 antibody
  • MDDGC5 antibody
  • zgc:162829 antibody

Protein level used designations for FKRP

fukutin related protein , fukutin-related protein , fukutin-related protein-like

GENE ID SPECIES
431512 Gallus gallus
484426 Canis lupus familiaris
539701 Bos taurus
571426 Danio rerio
716804 Macaca mulatta
746394 Pan troglodytes
100145309 Xenopus (Silurana) tropicalis
100356414 Oryctolagus cuniculus
100385568 Callithrix jacchus
100448398 Pongo abelii
100516858 Sus scrofa
100603615 Nomascus leucogenys
79147 Homo sapiens
243853 Mus musculus
308390 Rattus norvegicus
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