ABCD4 Antibody

Datasheet

Code	CSB-PA001075LA01HU
Size	US$166
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Image	IHC image of CSB-PA001075LA01HU diluted at 1:100 and staining in paraffin-embedded human pancreatic cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
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Product Details
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Target Background

Product Details

Full Product Name

Rabbit anti-Homo sapiens (Human) ABCD4 Polyclonal antibody

Uniprot No.

O14678

Target Names

ABCD4

Alternative Names

ABCD4; PXMP1L; Lysosomal cobalamin transporter ABCD4; ATP-binding cassette sub-family D member 4; PMP70-related protein; P70R; Peroxisomal membrane protein 1-like; PXMP1-L; Peroxisomal membrane protein 69; PMP69

Raised in

Rabbit

Species Reactivity

Human

Immunogen

Recombinant Human ATP-binding cassette sub-family D member 4 protein (335-606AA)

Immunogen Species

Homo sapiens (Human)

Conjugate

Non-conjugated

The ABCD4 Antibody (Product code: CSB-PA001075LA01HU) is Non-conjugated. For ABCD4 Antibody with conjugates, please check the following table.

Conjugate	Product Code	Product Name	Application
HRP	CSB-PA001075LB01HU	ABCD4 Antibody, HRP conjugated	ELISA
FITC	CSB-PA001075LC01HU	ABCD4 Antibody, FITC conjugated
Biotin	CSB-PA001075LD01HU	ABCD4 Antibody, Biotin conjugated	ELISA

Clonality

Polyclonal

Isotype

IgG

Purification Method

Antigen Affinity Purified

Concentration

It differs from different batches. Please contact us to confirm it.

Buffer

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4

Form

Liquid

Tested Applications

ELISA, IHC

Recommended Dilution

Application	Recommended Dilution
IHC	1:20-1:200

Protocols

ELISA Protocol
Immunohistochemistry (IHC) Protocol

Troubleshooting and FAQs

Antibody FAQs

Storage

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

Lead Time

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

Customer Reviews and Q&A

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Target Background

Function

Lysosomal membrane protein that transports cobalamin (Vitamin B12) from the lysosomal lumen to the cytosol in an ATP-dependent manner. Targeted by LMBRD1 lysosomal chaperone from the endoplasmic reticulum to the lysosomal membrane. Then forms a complex with lysosomal chaperone LMBRD1 and cytosolic MMACHC to transport cobalamin across the lysosomal membrane.

Gene References into Functions

endogenous ABCD4 was localized to both lysosomes and the ER, and its lysosomal localization was disturbed by knockout of LMBRD1 PMID: 27456980
Data suggest that ABCD4 lysosomal targeting depends on co-expression of and interaction with LMBRD1; mutations in LMBRD1 and ABCD4 that result in cobalamin metabolism disorders cblF and cblJ (or mutations in ATPase domain) disrupt interactions between LMBRD1 and ABCD4. (LMBRD1 = nuclear export signal-interacting protein; ABCD4 = ATP-binding cassette, sub-family D (ALD), member 4) PMID: 28572511
Results propose a model whereby membrane-bound LMBD1 and ABCD4 facilitate the vectorial delivery of lysosomal vitamin B12 to cytoplasmic MMACHC. PMID: 25535791
mutations altering the putative ATPase domain of ABCD4 affect its function, suggesting that the ATPase activity of ABCD4 may be involved in intracellular processing of vitamin B12. PMID: 22922874
Defects in ABCB4 have been found to cause progressive familial intrahepatic cholestasis type 3. PMID: 21514256
expression tends to be correlated with the severity of X-linked adrenoleukodystrophy PMID: 15800013

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Involvement in disease

Methylmalonic aciduria and homocystinuria type cblJ (MAHCJ)

Subcellular Location

Endoplasmic reticulum membrane; Multi-pass membrane protein. Lysosome membrane; Multi-pass membrane protein.

Protein Families

ABC transporter superfamily, ABCD family, Peroxisomal fatty acyl CoA transporter (TC 3.A.1.203) subfamily

Tissue Specificity

Ubiquitous.

Database Links

HGNC: 68

OMIM: 603214

KEGG: hsa:5826

STRING: 9606.ENSP00000349396

UniGene: Hs.94395

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