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AB314618

Recombinant Human FKTN Protein (His Tag)

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Recombinant Human FKTN Protein (His Tag) is a Human Full Length protein, in the 1 to 461 aa range, expressed in Escherichia coli, with >85%, suitable for SDS-PAGE.

대체 명칭 보기

FCMD, FKTN, Ribitol-5-phosphate transferase FKTN, Fukutin, Fukuyama-type congenital muscular dystrophy protein, Ribitol-5-phosphate transferase

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SDS-PAGE - Recombinant Human FKTN Protein (His Tag) (AB314618)
  • SDS-PAGE

Supplier Data

SDS-PAGE - Recombinant Human FKTN Protein (His Tag) (AB314618)

(Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.

주요 정보

Purity

>85% SDS-PAGE

발현 시스템

Escherichia coli

Tags

10x His tag N-Terminus

Applications

SDS-PAGE

applications

Biologically active

No

Accession

Animal free

Yes

Carrier free

No

Species

Human

Reconstitution

We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL. We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20°C/-80°C. Our default final concentration of glycerol is 50%.

보관 버퍼

pH: 7.4 - 8 Constituents: 6% Trehalose, 0.87% Sodium chloride, 0.24% Tris, 0.05% 2-Octadecoxyethanol

storage-buffer

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보완 제품

Primary Antibodies

AB248403

Anti-Fukutin antibody [EPR7913] - BSA and Azide free

RabMAb|Recombinant

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Fukutin antibody [EPR7913] - BSA and Azide free (AB248403)

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Reactivity 정보

{ "title": "Reactivity Data", "filters": { "stats": ["", "Reactivity", "Dilution Info", "Notes"] }, "values": { "SDS-PAGE": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" } } }
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서열 정보

[{"linker":null,"sequence":"MSRINKNVVLALLTLTSSAFLLFQLYYYKHYLSTKNGAGLSKSKGSRIGFDSTQWRAVKKFIMLTSNQNVPVFLIDPLILELINKNFEQVKNTSHGSTSQCKFFCVPRDFTAFALQYHLWKNEEGWFRIAENMGFQCLKIESKDPRLDGIDSLSGTEIPLHYICKLATHAIHLVVFHERSGNYLWHGHLRLKEHIDRKFVPFRKLQFGRYPGAFDRPELQQVTVDGLEVLIPKDPMHFVEEVPHSRFIECRYKEARAFFQQYLDDNTVEAVAFRKSAKELLQLAAKTLNKLGVPFWLSSGTCLGWYRQCNIIPYSKDVDLGIFIQDYKSDIILAFQDAGLPLKHKFGKVEDSLELSFQGKDDVKLDVFFFYEETDHMWNGGTQAKTGKKFKYLFPKFTLCWTEFVDMKVHVPCETLEYIEANYGKTWKIPVKTWDWKRSPPNVQPNGIWPISEWDEVIQLY","proteinLength":"Full Length","predictedMolecularWeight":"56.5 kDa","actualMolecularWeight":null,"aminoAcidEnd":461,"aminoAcidStart":1,"nature":"Recombinant","expressionSystem":"Escherichia coli","accessionNumber":"O75072","tags":[{"tag":"10x His","terminus":"N-Terminus"}]}]
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특성 및 보관 정보

제형
Lyophilized
Purification 테크닉
Ion exchange chromatography
배송 시 보관 조건
Blue Ice
적절한 단기 보관 기간
1-2 weeks
적절한 단기 보관 조건
+4°C
적절한 장기 보관 조건
-80°C
분주 정보
Add glycerol to a final volume of 50% for extra stability and aliquot
보관 정보
The product can be stored for up to 12 months
False
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추가 정보

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

Fukutin also known as FKRP or FKTN is a glycosyltransferase enzyme weighing approximately 55 kDa. This protein participates in the process of adding sugar chains to proteins primarily glycosylating α-dystroglycan. Fukutin is highly expressed in muscle tissues including skeletal and cardiac muscles and plays a role in the central nervous system. Its activity is important for the proper function of these tissues.
Biological function summary

Fukutin plays a fundamental role in maintaining the structure and function of muscle cells by participating in the glycosylation of α-dystroglycan. This modification allows α-dystroglycan to bind to extracellular matrix proteins. Fukutin operates as part of the dystrophin-glycoprotein complex which is essential for muscle integrity and stability. This complex anchors the cytoskeleton of a muscle cell to the surrounding extracellular matrix.

Pathways

This protein fukutin participates in the dystroglycan pathway affecting the integrity of muscle cells. It works closely with other proteins like LARGE1 which also modifies α-dystroglycan. Fukutin's activity ensures that α-dystroglycan has the necessary post-translational modifications to perform its function within the laminin-binding pathway linking the extracellular matrix to the cytoskeleton.

Alterations in fukutin mutations can lead to muscular dystrophies such as Fukuyama congenital muscular dystrophy and Walker-Warburg syndrome. In these conditions the fukutin-related glycosylation of α-dystroglycan is disrupted which impairs muscle cell stability and function. Other proteins like dystrophin and sarcoglycans associated with the dystrophin-glycoprotein complex also play roles in these disorders further linking fukutin to muscle disease mechanisms.
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일반 정보

기능

Catalyzes the transfer of a ribitol-phosphate from CDP-ribitol to the distal N-acetylgalactosamine 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) (PubMed : 26923585, PubMed : 27194101, PubMed : 29477842). This constitutes the first step in the formation of the ribitol 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 : 17034757, PubMed : 25279699, PubMed : 26923585, PubMed : 27194101, PubMed : 29477842). Required for normal location of POMGNT1 in Golgi membranes, and for normal POMGNT1 activity (PubMed : 17034757). May interact with and reinforce a large complex encompassing the outside and inside of muscle membranes (PubMed : 25279699). Could be involved in brain development (Probable).

서열 유사성

Belongs to the LicD transferase family.

Subcellular localisation

Nucleus

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제품 프로토콜

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타겟 정보

Catalyzes the transfer of a ribitol-phosphate from CDP-ribitol to the distal N-acetylgalactosamine 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) (PubMed : 26923585, PubMed : 27194101, PubMed : 29477842). This constitutes the first step in the formation of the ribitol 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 : 17034757, PubMed : 25279699, PubMed : 26923585, PubMed : 27194101, PubMed : 29477842). Required for normal location of POMGNT1 in Golgi membranes, and for normal POMGNT1 activity (PubMed : 17034757). May interact with and reinforce a large complex encompassing the outside and inside of muscle membranes (PubMed : 25279699). Could be involved in brain development (Probable).
See full target information FKTN
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Product promise

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