CHM Antibody, HRP conjugated (PACO25742)
- SKU:
- PACO25742
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Antibody type:
- Polyclonal
- Conjugation:
- HRP
Frequently bought together:
Description
抗体名: | CHM Antibody, HRP conjugated (PACO25742) |
抗体コード: | PACO25742 |
サイズ: | 50ug |
宿主種: | Rabbit |
申し込み: | ELISA |
推奨される希釈: | |
反応性: | Human |
免疫原: | Recombinant Human Rab proteins geranylgeranyltransferase component A 1 protein (1-653AA) |
憲法: | Liquid |
ストレージバッファ: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, PH 7.4 |
精製方法: | >95%, Protein G purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | HRP |
バックグラウンド: | Substrate-binding subunit of the Rab geranylgeranyltransferase (GGTase) complex. Binds unprenylated Rab proteins and presents the substrate peptide to the catalytic component B composed of RABGGTA and RABGGTB, and remains bound to it after the geranylgeranyl transfer reaction. The component A is thought to be regenerated by transferring its prenylated Rab back to the donor membrane. Besides, a pre-formed complex consisting of CHM and the Rab GGTase dimer (RGGT or component B) can bind to and prenylate Rab proteins; this alternative pathway is proposed to be the predominant pathway for Rab protein geranylgeranylation. |
シノニム: | Rab proteins geranylgeranyltransferase component A 1 (Choroideremia protein) (Rab escort protein 1) (REP-1) (TCD protein), CHM, REP1 TCD |
UniProt Protein Function: | CHM: Binds unprenylated Rab proteins, presents it to the catalytic Rab GGTase dimer, and remains bound to it after the geranylgeranyl transfer reaction. The component A is thought to be regenerated by transferring its prenylated Rab back to the donor membrane. Also a pre-formed complex consisting of CHM and the Rab GGTase dimer (RGGT or component B) can bind to and prenylate Rab proteins; this alternative pathway is proposed to be the predominant pathway for Rab protein geranylgeranylation. Defects in CHM are the cause of choroideremia (CHM). An X-linked recessive disease characterized by a slowly progressive degeneration of the choroid, photoreceptors, and retinal pigment epithelium. Affected males develop night blindness in their teenage years followed by loss of peripheral vision and complete blindness at middle age. Carrier females are generally asymptomatic but funduscopic examination often shows patchy areas of chorioretinal atrophy. Belongs to the Rab GDI family. 2 isoforms of the human protein are produced by alternative splicing. |
UniProt Protein Details: | Protein type:Transferase Chromosomal Location of Human Ortholog: Xq21.2 Cellular Component: cytosol; Rab-protein geranylgeranyltransferase complex Molecular Function:Rab GTPase binding; Rab-protein geranylgeranyltransferase activity Biological Process: post-translational protein modification; protein geranylgeranylation; protein targeting to membrane; regulation of apoptosis Disease: Choroideremia |
NCBI Summary: | This gene encodes component A of the RAB geranylgeranyl transferase holoenzyme. In the dimeric holoenzyme, this subunit binds unprenylated Rab GTPases and then presents them to the catalytic Rab GGTase subunit for the geranylgeranyl transfer reaction. Rab GTPases need to be geranylgeranyled on either one or two cysteine residues in their C-terminus to localize to the correct intracellular membrane. Mutations in this gene are a cause of choroideremia; also known as tapetochoroidal dystrophy (TCD). This X-linked disease is characterized by progressive dystrophy of the choroid, retinal pigment epithelium and retina. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Mar 2016] |
UniProt Code: | P24386 |
NCBI GenInfo Identifier: | 9966761 |
NCBI Gene ID: | 1121 |
NCBI Accession: | NP_000381.1 |
UniProt Secondary Accession: | P24386,O43732, A1L4D2, |
UniProt Related Accession: | P24386 |
Molecular Weight: | 77.5 kDa |
NCBI Full Name: | rab proteins geranylgeranyltransferase component A 1 isoform a |
NCBI Synonym Full Names: | CHM, Rab escort protein 1 |
NCBI Official Symbol: | CHM |
NCBI Official Synonym Symbols: | TCD; GGTA; REP-1; DXS540; HSD-32 |
NCBI Protein Information: | rab proteins geranylgeranyltransferase component A 1 |
UniProt Protein Name: | Rab proteins geranylgeranyltransferase component A 1 |
UniProt Synonym Protein Names: | Choroideremia protein; Rab escort protein 1; REP-1; TCD protein |
Protein Family: | Rab proteins geranylgeranyltransferase component |
UniProt Gene Name: | CHM |