HPA003231
Anti-CHM antibody produced in rabbit
Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution
동의어(들):
Anti-Choroideraemia protein antibody produced in rabbit, Anti-REP-1 antibody produced in rabbit, Anti-Rab escort protein 1 antibody produced in rabbit, Anti-Rab proteins geranylgeranyltransferase component A 1 antibody produced in rabbit, Anti-TCD protein antibody produced in rabbit
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모든 사진(3)
About This Item
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
immunohistochemistry: 1:200- 1:500
면역원 서열
SRKDKTIQHVEVFCYASQDLHEDVEEAGALQKNHALVTSANSTEAADSAFLPTEDESLSTMSCEMLTEQTPSSDPENALEVNGAEVTGEKENHCDDKTCVPSTSAEDMSENVPIAEDTTEQPKKNRITYSQIIKEG
UniProt 수납 번호
배송 상태
wet ice
저장 온도
−20°C
타겟 번역 후 변형
unmodified
유전자 정보
human ... CHM(1121)
일반 설명
The gene CHM (choroideremia) is mapped to human chromosome Xq21.2. It encodes a 653 amino acid protein. It contains 15 exons spanning a length of 150kb.
면역원
Rab proteins geranylgeranyltransferase component A 1 recombinant protein epitope signature tag (PrEST)
애플리케이션
Anti-CHM antibody produced in rabbit, a Prestige Antibody, 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. The antibodies are also tested using immunofluorescence and western blotting. To view these protocols and other useful information about Prestige Antibodies and the HPA, visit sigma.com/prestige.
생화학적/생리학적 작용
CHM (choroideremia) gene encodes a protein that is also called as Rab proteins geranylgeranyltransferase component A 1. This subunit is a substrate-binding subunit of the Rab geranylgeranyl transferase (RGGT) holoenzyme. It binds unprenylated Rab proteins and presents them to the catalytic component B of Rab-GGTase. Alternatively, the subunit encoded by this gene first binds to RGGT, and this complex then binds and prenylates Rab proteins. These Rab proteins are small GTPases that function in regulating vesicular membrane traffic. Mutations in this gene can cause choroideremia, also referred to as tapetochoroidal dystrophy (TCD), that is characterized by progressive dystrophy of the choroid, retinal pigment epithelium and retina.
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 APREST74342
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.
Kanmin Xue et al.
Nature medicine, 24(10), 1507-1512 (2018-10-10)
Retinal gene therapy is increasingly recognized as a novel molecular intervention that has huge potential in treating common causes of blindness, the majority of which have a genetic aetiology1-5. Choroideremia is a chronic X-linked retinal degeneration that was first described
J A van den Hurk et al.
Human mutation, 9(2), 110-117 (1997-01-01)
Choroideremia (CHM) is an X-linked recessive eye disease that results from mutations involving the Rab escort protein-1 (REP-1) gene. In 18 patients deletions of different sizes have been found. Two females suffering from CHM were reported to have translocations that
K Alexandrov et al.
The EMBO journal, 13(22), 5262-5273 (1994-11-15)
Rab proteins comprise a family of small GTPases that serve a regulatory role in vesicular membrane traffic. Geranylgeranylation of these proteins on C-terminal cysteine motifs is crucial for their membrane association and function. This post-translational modification is catalysed by rab
Rudi A Baron et al.
The Biochemical journal, 415(1), 67-75 (2008-06-06)
Prenylation (or geranylgeranylation) of Rab GTPases is catalysed by RGGT (Rab geranylgeranyl transferase) and requires REP (Rab escort protein). In the classical pathway, REP associates first with unprenylated Rab, which is then prenylated by RGGT. In the alternative pathway, REP
Aaron Black et al.
The journal of gene medicine, 16(5-6), 122-130 (2014-06-26)
Choroideremia (CHM) is a slowly progressive X-linked retinal degeneration that results in a loss of photoreceptors, retinal pigment epithelium and choroid. CHM, the gene implicated in choroideremia, encodes Rab escort protein-1 (REP-1), which is involved in the post-translational activation via
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