Signal Transduction Antibodies 3

Anti-CHM Antibody (CAB8345)

(No reviews yet) Write a Review
SKU:
CAB8345
Product Type:
Antibody
Applications:
WB
Reactivity:
Human
Host Species:
Rabbit
Isotype:
IgG
Research Area:
Signal Transduction

Description

system_update_altDatasheetsystem_update_altMSDS
Antibody Name:Anti-CHM Antibody
Antibody SKU:CAB8345
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Human
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 120-210 of human CHM (NP_000381.1).
Application:WB
Recommended Dilution:WB 1:500 - 1:2000
Reactivity:Human
Positive Samples:U-251MG, LO2, NCI-H460, HeLa
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 120-210 of human CHM (NP_000381.1).
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:KNHA LVTS ANST EAAD SAFL PTED ESLS TMSC EMLT EQTP SSDP ENAL EVNG AEVT GEKE NHCD DKTC VPST SAED MSEN VPIA EDTT EQP
Gene ID:1121
Uniprot:P24386
Cellular Location:Cytoplasm, cytosol
Calculated MW:12kDa/73kDa
Observed MW:101kDa
Synonyms:CHM, DXS540, GGTA, HSD-32, REP-1, TCD
Background: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.
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  
Anti-CHM Polyclonal Antibody (CAB8345)Western blot analysis of extracts of various cell lines, using CHM antibody (CAB8345) at 1:1000 dilution._Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution._Lysates/proteins: 25ug per lane._Blocking buffer: 3% nonfat dry milk in TBST._Detection: ECL Enhanced Kit (RM00021)._Exposure time: 5s.
View AllClose

0 Reviews

View AllClose

Related Products

Anti-CHM Antibody (CAB9378)

Anti-CHM Antibody (CAB9378)

OverviewAntibody Name:Anti-CHM Antibody (CAB9378)Antibody SKU:CAB9378Antibody Size:50µL, 100µLApplication:Western blottingReactivity:</