Signal Transduction Antibodies 3

Anti-ABCC6 Antibody (CAB8100)

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SKU:
CAB8100
Product Type:
Antibody
Applications:
WB
Reactivity:
Mouse
Reactivity:
Rat
Host Species:
Rabbit
Isotype:
IgG
Research Area:
Signal Transduction

Description

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Antibody Name:ABCC6 Rabbit Polyclonal Antibody
Antibody SKU:CAB8100
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Mouse, Rat
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 830-940 of human ABCC6 (NP_001162.4).
Application:WB
Recommended Dilution:WB 1:500 - 1:2000
Reactivity:Mouse, Rat
Positive Samples:mouse liver, rat liver
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 830-940 of human ABCC6 (NP_001162.4).
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:AIAE MGSY QELL QRKG ALVC LLDQ ARQP GDRG EGET EPGT STKD PRGT SAGR RPEL RRER SIKS VPEK DRTT SEAQ TEVP LDDP DRAG WPAG KDSI QYGR VKAT VHLA YLR
Gene ID:368
Uniprot:O95255
Cellular Location:Basolateral cell membrane, Endoplasmic reticulum membrane, Multi-pass membrane protein, Single-pass membrane protein
Calculated MW:10kDa/95kDa/164kDa
Observed MW:164kDa
Synonyms:ABCC6, ABC34, ARA, EST349056, GACI2, MLP1, MOAT-E, MOATE, MRP6, PXE, PXE1, URG7
Background:The protein encoded by this gene is a member of the superfamily of ATP-binding cassette (ABC) transporters. ABC proteins transport various molecules across extra- and intra-cellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). The encoded protein, a member of the MRP subfamily, is involved in multi-drug resistance. Mutations in this gene cause pseudoxanthoma elasticum. Alternatively spliced transcript variants that encode different proteins have been described for this gene.
UniProt Protein Function:ABCC6: May participate directly in the active transport of drugs into subcellular organelles or influence drug distribution indirectly. Transports glutathione conjugates as leukotriene-c4 (LTC4) and N-ethylmaleimide S-glutathione (NEM-GS). Defects in ABCC6 are the cause of pseudoxanthoma elasticum (PXE). PXE is a disorder characterized by calcification of elastic fibers in skin, arteries and retina that results in dermal lesions with associated laxity and loss of elasticity, arterial insufficiency and retinal hemorrhages leading to macular degeneration. PXE is caused in the overwhelming majority of cases by homozygous or compound heterozygous mutations in the ABCC6 gene (autosomal recessive PXE). Individuals carrying heterozygous mutations express limited manifestations of the pseudoxanthoma elasticum phenotype (autosomal dominant PXE). Defects in ABCC6 are the cause of arterial calcification of infancy, generalized, type 2 (GACI2). GACI2 is a severe autosomal recessive disorder characterized by calcification of the internal elastic lamina of muscular arteries and stenosis due to myointimal proliferation. The disorder is often fatal within the first 6 months of life because of myocardial ischemia resulting in refractory heart failure. Belongs to the ABC transporter superfamily. ABCC family. Conjugate transporter (TC 3.A.1.208) subfamily.
UniProt Protein Details:

Protein type:Hydrolase; Membrane protein, integral; Membrane protein, multi-pass; Transporter; Transporter, ABC family

Chromosomal Location of Human Ortholog: 16p13.11

Cellular Component: nucleus; plasma membrane

Molecular Function:anion transmembrane-transporting ATPase activity; ATPase activity, coupled to transmembrane movement of substances; transporter activity

Biological Process: response to drug; transmembrane transport; transport

Disease: Arterial Calcification, Generalized, Of Infancy, 2; Pseudoxanthoma Elasticum; Pseudoxanthoma Elasticum, Forme Fruste

NCBI Summary:The protein encoded by this gene is a member of the superfamily of ATP-binding cassette (ABC) transporters. ABC proteins transport various molecules across extra- and intra-cellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). The encoded protein, a member of the MRP subfamily, is involved in multi-drug resistance. Mutations in this gene cause pseudoxanthoma elasticum. Alternatively spliced transcript variants that encode different proteins have been described for this gene. [provided by RefSeq, Jul 2008]
UniProt Code:O95255
NCBI GenInfo Identifier:118582251
NCBI Gene ID:368
NCBI Accession:NP_001072996.1
UniProt Secondary Accession:O95255,P78420, Q8TCY8, Q9UMZ7, A2RRN8, A8KIG6, A8Y988 E7ESW8,
UniProt Related Accession:O95255
Molecular Weight:165 kDa
NCBI Full Name:URG7 protein isoform 2
NCBI Synonym Full Names:ATP binding cassette subfamily C member 6
NCBI Official Symbol:ABCC6  
NCBI Official Synonym Symbols:ARA; PXE; MLP1; MRP6; PXE1; URG7; ABC34; GACI2; MOATE; MOAT-E; EST349056  
NCBI Protein Information:multidrug resistance-associated protein 6; URG7 protein
UniProt Protein Name:Multidrug resistance-associated protein 6
UniProt Synonym Protein Names:ATP-binding cassette sub-family C member 6; Anthracycline resistance-associated protein; Multi-specific organic anion transporter E; MOAT-E
UniProt Gene Name:ABCC6  
Anti-ABCC6 Polyclonal Antibody (CAB8100)Western blot analysis of extracts of various cell lines, using ABCC6 antibody (CAB8100) 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 Basic Kit (RM00020). Exposure time: 30s.
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