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AB153505

Recombinant Human FKRP protein (GST tag N-Terminus)

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Recombinant Human FKRP protein (GST tag N-Terminus) is a Human Fragment protein, in the 396 to 494 aa range, expressed in Wheat germ, suitable for ELISA, WB.

View Alternative Names

Ribitol 5-phosphate transferase FKRP, Fukutin-related protein, Ribitol-5-phosphate transferase, FKRP

1 Images
SDS-PAGE - Recombinant Human FKRP protein (GST tag N-Terminus) (AB153505)
  • SDS-PAGE

Unknown

SDS-PAGE - Recombinant Human FKRP protein (GST tag N-Terminus) (AB153505)

ab153505 on a 12.5% SDS-PAGE stained with Coomassie Blue.

Key facts

Expression system

Wheat germ

Tags

GST tag N-Terminus

Applications

ELISA, WB

applications

Biologically active

No

Accession

Q9H9S5

Animal free

No

Carrier free

No

Species

Human

Storage buffer

pH: 8 Constituents: 0.79% Tris HCl, 0.31% Glutathione

storage-buffer

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Complementary Products

Primary Antibodies

AB65243

Anti-FKRP antibody

Western blot - Anti-FKRP antibody (AB65243)

  • 0
  • 0
Primary Antibodies

AB11576

Anti-Laminin 2 alpha antibody [4H8-2]

Immunohistochemistry (Frozen sections) - Anti-Laminin 2 alpha antibody [4H8-2] (AB11576)

  • 5
  • 8
Primary Antibodies

AB22595

Anti-Calnexin antibody - ER Marker

Lab Essentials|KO Validated

Immunocytochemistry/ Immunofluorescence - Anti-Calnexin antibody - ER Marker (AB22595)

  • 5
  • 28
Primary Antibodies

AB191881

Anti-Lin28B antibody [EPR18717]

RabMAb|Recombinant|KO Validated

Flow Cytometry (Intracellular) - Anti-Lin28B antibody [EPR18717] (AB191881)

  • 0
  • 0
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Reactivity data

{ "title": "Reactivity Data", "filters": { "stats": ["", "Reactivity", "Dilution Info", "Notes"] }, "values": { "ELISA": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" }, "WB": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" } } }
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Sequence info

[{"sequence":"KAVEGDFFRVQYSESNHLHVDLWPFYPRNGVMTKDTWLDHRQDVEFPEHFLQPLVPLPFAGFVAQAPNNYRRFLELKFGPGVIENPQYPNPALLSLTGS","proteinLength":"Fragment","predictedMolecularWeight":null,"actualMolecularWeight":null,"aminoAcidEnd":494,"aminoAcidStart":396,"nature":"Recombinant","expressionSystem":"Wheat germ","accessionNumber":"Q9H9S5","tags":[{"tag":"GST","terminus":"N-Terminus"}]}]
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Properties and storage information

Shipped at conditions
Dry Ice
Appropriate short-term storage conditions
-80°C
Appropriate long-term storage conditions
-80°C
Aliquoting information
Upon delivery aliquot
Storage information
Avoid freeze / thaw cycle
False
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Supplementary information

This supplementary information is collated from multiple sources and compiled automatically.

The FKRP protein short for fukutin-related protein plays an important role in the glycosylation of alpha-dystroglycan an important component of muscle tissue structure. FKRP weighs approximately 55 kDa and is present in several tissues with high expression levels found in skeletal and cardiac muscle. It resides mainly within the Golgi apparatus where it participates in the modification of glycoproteins.
Biological function summary

FKRP functions as a glycosyltransferase involved in the synthesis of O-linked mannose structures on alpha-dystroglycan. Alpha-dystroglycan forms part of the dystrophin-glycoprotein complex a critical assembly in muscle tissue for linking the extracellular matrix to the cytoskeleton. This connection facilitates cellular stability during muscle contraction and maintains tissue integrity.

Pathways

FKRP operates predominantly within the alpha-dystroglycan glycosylation pathway. This pathway ensures proper glycan structures are present on alpha-dystroglycan permitting its interaction with extracellular matrix components like laminin. FKRP's activity closely interacts with other proteins like fukutin and LARGE1 which also participate in the modification of alpha-dystroglycan therefore impacting overall cell-matrix interactions.

FKRP mutations can lead to muscular dystrophies such as Walker-Warburg syndrome and limb-girdle muscular dystrophy 2I. These disorders often result from deficient glycosylation of alpha-dystroglycan leading to compromised muscle function and stability. Variations in FKRP expression or function affect the dystrophin-glycoprotein complex and consequently influence the muscle tissue's ability to withstand mechanical stress implicating FKRP as a critical player in maintaining muscular health.
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Specifications

Form

Liquid

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General info

Function

Catalyzes the transfer of a ribitol 5-phosphate from CDP-L-ribitol to the ribitol 5-phosphate previously attached by FKTN/fukutin to the phosphorylated O-mannosyl trisaccharide (N-acetylgalactosamine-beta-3-N-acetylglucosamine-beta-4-(phosphate-6-)mannose), a carbohydrate structure present in alpha-dystroglycan (DAG1) (PubMed : 26923585, PubMed : 27194101, PubMed : 29477842, PubMed : 31949166). This constitutes the second step in the formation of the ribose 5-phosphate tandem repeat which links the phosphorylated O-mannosyl trisaccharide to the ligand binding moiety composed of repeats of 3-xylosyl-alpha-1,3-glucuronic acid-beta-1 (PubMed : 25279699, PubMed : 26923585, PubMed : 27194101, PubMed : 29477842, PubMed : 31949166).

Sequence similarities

Belongs to the LicD transferase family.

Post-translational modifications

N-glycosylated.

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Product protocols

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Target data

Catalyzes the transfer of a ribitol 5-phosphate from CDP-L-ribitol to the ribitol 5-phosphate previously attached by FKTN/fukutin to the phosphorylated O-mannosyl trisaccharide (N-acetylgalactosamine-beta-3-N-acetylglucosamine-beta-4-(phosphate-6-)mannose), a carbohydrate structure present in alpha-dystroglycan (DAG1) (PubMed : 26923585, PubMed : 27194101, PubMed : 29477842, PubMed : 31949166). This constitutes the second step in the formation of the ribose 5-phosphate tandem repeat which links the phosphorylated O-mannosyl trisaccharide to the ligand binding moiety composed of repeats of 3-xylosyl-alpha-1,3-glucuronic acid-beta-1 (PubMed : 25279699, PubMed : 26923585, PubMed : 27194101, PubMed : 29477842, PubMed : 31949166).
See full target information FKRP
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