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Merck

HPA003231

Sigma-Aldrich

Anti-CHM antibody produced in rabbit

Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution

Synonym(e):

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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About This Item

UNSPSC-Code:
12352203
Human Protein Atlas-Nummer:
NACRES:
NA.41

Biologische Quelle

rabbit

Qualitätsniveau

Konjugat

unconjugated

Antikörperform

affinity isolated antibody

Antikörper-Produkttyp

primary antibodies

Klon

polyclonal

Produktlinie

Prestige Antibodies® Powered by Atlas Antibodies

Form

buffered aqueous glycerol solution

Speziesreaktivität

human

Methode(n)

immunohistochemistry: 1:200- 1:500

Immunogene Sequenz

SRKDKTIQHVEVFCYASQDLHEDVEEAGALQKNHALVTSANSTEAADSAFLPTEDESLSTMSCEMLTEQTPSSDPENALEVNGAEVTGEKENHCDDKTCVPSTSAEDMSENVPIAEDTTEQPKKNRITYSQIIKEG

UniProt-Hinterlegungsnummer

Versandbedingung

wet ice

Lagertemp.

−20°C

Posttranslationale Modifikation Target

unmodified

Angaben zum Gen

human ... CHM(1121)

Allgemeine Beschreibung

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)

Anwendung

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. Wirkung

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.

Leistungsmerkmale und Vorteile

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:
  • 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.

Verlinkung

Corresponding Antigen APREST74342

Physikalische Form

Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide

Rechtliche Hinweise

Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany

Haftungsausschluss

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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Lagerklassenschlüssel

10 - Combustible liquids

WGK

WGK 1

Flammpunkt (°F)

Not applicable

Flammpunkt (°C)

Not applicable

Persönliche Schutzausrüstung

Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)


Analysenzertifikate (COA)

Suchen Sie nach Analysenzertifikate (COA), indem Sie die Lot-/Chargennummer des Produkts eingeben. Lot- und Chargennummern sind auf dem Produktetikett hinter den Wörtern ‘Lot’ oder ‘Batch’ (Lot oder Charge) zu finden.

Besitzen Sie dieses Produkt bereits?

In der Dokumentenbibliothek finden Sie die Dokumentation zu den Produkten, die Sie kürzlich erworben haben.

Die Dokumentenbibliothek aufrufen

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
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
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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