HPA000166
Anti-DKC1 antibody produced in rabbit
Ab1, Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution
동의어(들):
Anti-CBF5 homolog antibody produced in rabbit, Anti-Dyskerin antibody produced in rabbit, Anti-H/ACA ribonucleoprotein complex subunit 4 antibody produced in rabbit, Anti-Nopp140-associated protein of 57 kDa antibody produced in rabbit, Anti-Nucleolar protein NAP57 antibody produced in rabbit, Anti-Nucleolar protein family A member 4 antibody produced in rabbit, Anti-snoRNP protein DKC1 antibody produced in rabbit
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모든 사진(5)
About This Item
UNSPSC 코드:
12352203
인간 단백질 도해서 번호:
추천 제품
생물학적 소스
rabbit
Quality Level
결합
unconjugated
항체 형태
affinity isolated antibody
항체 생산 유형
primary antibodies
클론
polyclonal
제품 라인
Prestige Antibodies® Powered by Atlas Antibodies
형태
buffered aqueous glycerol solution
종 반응성
human
기술
immunoblotting: 0.04-0.4 μg/mL
immunohistochemistry: 1:50-1:200
면역원 서열
EAGTYIRTLCVHLGLLLGVGGQMQELRRVRSGVMSEKDHMVTMHDVLDAQWLYDNHKDESYLRRVVYPLEKLLTSHKRLVMKDSAVNAICYGAKIMLPGVLRYEDGIEVNQEIVVIT
UniProt 수납 번호
배송 상태
wet ice
저장 온도
−20°C
타겟 번역 후 변형
unmodified
유전자 정보
human ... DKC1(1736)
유사한 제품을 찾으십니까? 방문 제품 비교 안내
일반 설명
Dyskeratosis congenita 1 (DKC1) gene encodes dyskerin, which is a 514-amino-acid protein. This gene consists of 15 exons comprising a region of 15kb, with the internal exons size range of 65-185bp.
면역원
H/ACA ribonucleoprotein complex subunit 4 recombinant protein epitope signature tag (PrEST)
애플리케이션
All Prestige Antibodies®Powered by Atlas Antibodies is developed and validated by the Human Protein Atlas (HPA) project . Each antibody is tested by immunohistochemistry against hundreds of normal and disease tissues. These images can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. We also provide Prestige Antibodies® protocols and other useful information.
생화학적/생리학적 작용
Mutation in the DKC1 (Dyskeratosis congenita 1) gene will lead to the X-linked form of the disease called as Dyskeratosis congenita (DKC), a rare inherited bone marrow-failure syndrome. DKC is characterised by multiple features including nail dystrophy, abnormal skin pigmentation and increased susceptibility to cancer. Altered DKC1 gene will cause depletion in bone marrow production which is one of the major causes for premature death. DKC is a nucleolar protein with multiple roles including centromere function, rRNA transcription and nucleocytoplasmic trafficking etc.
특징 및 장점
Prestige Antibodies® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.
Every Prestige Antibody is tested in the following ways:
Every Prestige Antibody is tested in the following ways:
- IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
- Protein array of 364 human recombinant protein fragments.
결합
Corresponding Antigen APREST74148
물리적 형태
Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide
법적 정보
Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany
면책조항
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
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Storage Class Code
10 - Combustible liquids
WGK
WGK 1
Flash Point (°F)
Not applicable
Flash Point (°C)
Not applicable
개인 보호 장비
Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)
시험 성적서(COA)
제품의 로트/배치 번호를 입력하여 시험 성적서(COA)을 검색하십시오. 로트 및 배치 번호는 제품 라벨에 있는 ‘로트’ 또는 ‘배치’라는 용어 뒤에서 찾을 수 있습니다.
Josu de la Fuente et al.
Pediatric transplantation, 11(6), 584-594 (2007-08-01)
DC is a multisystem bone marrow failure syndrome exhibiting marked clinical and genetic heterogeneity. X-linked, autosomal dominant and autosomal recessive subtypes are recognized. The gene mutated in X-linked DC (DKC1) encodes a highly conserved nucleolar protein called dyskerin. Dyskerin associates
M Kirwan et al.
Clinical genetics, 73(2), 103-112 (2007-11-17)
Dyskeratosis congenita (DC) is an inherited syndrome exhibiting marked clinical and genetic heterogeneity. It is characterized by multiple features including mucocutaneous abnormalities, bone marrow failure and an increased predisposition to cancer. Three genetic subtypes are recognized: X-linked recessive DC bears
P Sieron et al.
British journal of cancer, 101(8), 1410-1416 (2009-09-17)
Dyskerin encoded by the DKC1 gene is a predominantly nucleolar protein essential for the formation of pseudouridine in RNA and the telomerase RNA subunit hTR. Inherited mutations inactivating dyskerin cause dyskeratosis congenita, a syndrome with progeroid features characterised by skin
Lorenzo Montanaro et al.
Cellular oncology : the official journal of the International Society for Cellular Oncology, 30(6), 483-490 (2008-10-22)
The nucleolar protein dyskerin is involved in the modification of specific uridine residues to pseudouridine on ribosomal and small nuclear RNAs and in the stabilization of the telomerase RNA component (TERC). In this study we investigated for the first time
S W Knight et al.
American journal of human genetics, 65(1), 50-58 (1999-06-12)
Dyskeratosis congenita is a rare inherited bone marrow-failure syndrome characterized by abnormal skin pigmentation, nail dystrophy, and mucosal leukoplakia. More than 80% of patients develop bone-marrow failure, and this is the major cause of premature death. The X-linked form of
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