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AB238583

Anti-FKRP antibody

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Rabbit Polyclonal FKRP antibody. Suitable for IHC-P and reacts with Human samples. Cited in 1 publication. Immunogen corresponding to Recombinant Fragment Protein within Human FKRP aa 1-150.

대체 명칭 보기

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

2 이미지
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-FKRP antibody (AB238583)
  • IHC-P

Supplier Data

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-FKRP antibody (AB238583)

Paraffin-embedded human ovarian cancer tissue stained for FKRP using ab238583 at 1/400 dilution in immunohistochemical analysis.

After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30 minutes at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-FKRP antibody (AB238583)
  • IHC-P

Supplier Data

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-FKRP antibody (AB238583)

Paraffin-embedded human placenta tissue stained for FKRP using ab238583 at 1/400 dilution in immunohistochemical analysis.

After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30 minutes at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.

주요 정보

Host species

Rabbit

Clonality

Polyclonal

Isotype

IgG

Carrier free

No

Reacts with

Human

Applications

IHC-P

applications

Immunogen

Recombinant Fragment Protein within Human FKRP aa 1-150. The exact immunogen used to generate this antibody is proprietary information.

Q9H9S5

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대체품

Primary Antibodies

AB220059

Anti-FKRP antibody

Immunocytochemistry/ Immunofluorescence - Anti-FKRP antibody (AB220059)

  • 0
  • 0
Primary Antibodies

AB65243

Anti-FKRP antibody

Western blot - Anti-FKRP antibody (AB65243)

  • 0
  • 0

보완 제품

Primary Antibodies

AB204676

Alexa Fluor® 647 Anti-non-muscle Myosin IIA antibody [EPR8965]

RabMAb|Recombinant

Immunocytochemistry/ Immunofluorescence - Alexa Fluor® 647 Anti-non-muscle Myosin IIA antibody [EPR8965] (AB204676)

  • 0
  • 0
Primary Antibodies

AB92738

Anti-CGA/hCG alpha antibody [EP3373]

BOND RX™ Validated|RabMAb|Recombinant

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-CGA/hCG alpha antibody [EP3373] (AB92738)

  • 0
  • 0
Primary Antibodies

AB68807

Anti-GIYD2 antibody

Western blot - Anti-GIYD2 antibody (AB68807)

  • 0
  • 0
Primary Antibodies

AB139403

Anti-alpha 1 Spectrin antibody [EPR9300]

RabMAb|Recombinant

Flow Cytometry (Intracellular) - Anti-alpha 1 Spectrin antibody [EPR9300] (AB139403)

  • 0
  • 0
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Reactivity 정보

{ "title": "Reactivity Data", "filters": { "stats": ["", "Species", "Dilution Info", "Notes"], "tabs": { "all-applications": {"fullname" : "All Applications", "shortname": "All Applications"}, "IHCP" : {"fullname" : "Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)", "shortname":"IHC-P"} }, "product-promise": { "all": "all", "testedAndGuaranteed": "tested", "guaranteed": "expected", "predicted": "predicted", "notRecommended": "not-recommended" } }, "values": { "Human": { "IHCP-species-checked": "testedAndGuaranteed", "IHCP-species-dilution-info": "1/200 - 1/500", "IHCP-species-notes": "<p></p>" }, "Mouse": { "IHCP-species-checked": "predicted", "IHCP-species-dilution-info": "", "IHCP-species-notes": "" } } }
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특성 및 보관 정보

제형
Liquid
Purification 테크닉
Affinity purification Protein G
Purification 관련 사항
Purity >95%
보관 버퍼
pH: 7.4 Preservative: 0.03% Proclin 300 Constituents: PBS, 50% Glycerol (glycerin, glycerine)
배송 시 보관 조건
Blue Ice
적절한 단기 보관 기간
1-2 weeks
적절한 단기 보관 조건
+4°C
적절한 장기 보관 조건
-20°C
분주 정보
Upon delivery aliquot
보관 정보
Avoid freeze / thaw cycle
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추가 정보

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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제품 프로토콜

For this product, it's our understanding that no specific protocols are required. You can visit:
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타겟 정보

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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제품이 사용된 논문 (1)

Recent publications for all applications. Explore the 전체 목록 and refine your search

The Journal of clinical investigation 134: PubMed38085578

2023

Itaconate suppresses atherosclerosis by activating a Nrf2-dependent antiinflammatory response in macrophages in mice.

Applications

Unspecified application

Species

Unspecified reactive species

Jianrui Song,Yanling Zhang,Ryan A Frieler,Anthony Andren,Sherri Wood,Daniel J Tyrrell,Peter Sajjakulnukit,Jane C Deng,Costas A Lyssiotis,Richard M Mortensen,Morgan Salmon,Daniel R Goldstein
제품이 사용된 논문 모두 보기
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Product promise

저희는 고품질 시약으로 고객님의 연구를 서포트하고자 최선을 다하고 있으며, 모든 단계에서 함께 하겠습니다. 만약 제품이 기대한 성능을 보이지 않을 경우에도 Product Promise로 보호받으실 수 있습니다.
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