HPA003231
Anti-CHM antibody produced in rabbit
Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution
别名:
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
产品名称
Anti-CHM antibody produced in rabbit, Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution
biological source
rabbit
conjugate
unconjugated
antibody form
affinity isolated antibody
antibody product type
primary antibodies
clone
polyclonal
product line
Prestige Antibodies® Powered by Atlas Antibodies
form
buffered aqueous glycerol solution
species reactivity
human
technique(s)
immunohistochemistry: 1:200- 1:500
immunogen sequence
SRKDKTIQHVEVFCYASQDLHEDVEEAGALQKNHALVTSANSTEAADSAFLPTEDESLSTMSCEMLTEQTPSSDPENALEVNGAEVTGEKENHCDDKTCVPSTSAEDMSENVPIAEDTTEQPKKNRITYSQIIKEG
UniProt accession no.
shipped in
wet ice
storage temp.
−20°C
target post-translational modification
unmodified
Quality Level
Gene Information
human ... CHM(1121)
Application
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.
Biochem/physiol Actions
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.
Disclaimer
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.
Features and Benefits
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.
General description
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.
Immunogen
Rab proteins geranylgeranyltransferase component A 1 recombinant protein epitope signature tag (PrEST)
Other Notes
Corresponding Antigen APREST74342
Physical form
Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide
Legal Information
Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany
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存储类别
10 - Combustible liquids
wgk
WGK 1
flash_point_f
Not applicable
flash_point_c
Not applicable
ppe
Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)
法规信息
常规特殊物品
此项目有
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
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
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
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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