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AB153505

Recombinant Human FKRP protein (GST tag N-Terminus)

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.

別名を表示する

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

発現系

Wheat germ

タグ

GST tag N-Terminus

アプリケーション

ELISA, WB

applications

生物活性

No

アクセッション番号

Q9H9S5

アニマルフリー

No

キャリアフリー

No

種

Human

バッファー組成

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

storage-buffer

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組合せ製品

Proteins & Peptides

AB163270

Recombinant Human CMAS protein (GST tag N-Terminus)

SDS-PAGE - Recombinant Human CMAS protein (GST tag N-Terminus) (AB163270)

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Primary Antibodies

AB65243

Anti-FKRP antibody

Western blot - Anti-FKRP antibody (AB65243)

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Proteins & Peptides

AB229367

Recombinant Human Mannan Binding Lectin/MBL protein (His tag C-Terminus)

SDS-PAGE - Recombinant Human Mannan Binding Lectin/MBL protein (His tag C-Terminus) (AB229367)

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Proteins & Peptides

AB159814

Recombinant Human Wnt9a protein (GST tag N-Terminus)

SDS-PAGE - Recombinant Human Wnt9a protein (GST tag N-Terminus) (AB159814)

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  • 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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配列情報

[{"linker":null,"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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出荷温度及び保存条件

製品の状態
Liquid
精製方法
Proprietary technique
出荷温度
Dry Ice
短期保存温度
-80°C
長期保存温度
-80°C
分注に関する情報
Upon delivery aliquot
保管に関する情報
Avoid freeze / thaw cycle
False
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補足情報

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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一般的な情報

機能

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).

配列の類似性

Belongs to the LicD transferase family.

翻訳後修飾

N-glycosylated.

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製品プロトコール

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ターゲットの情報

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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