Home > Product > Antibody > Rabbit Anti-ABCA3/P36 Lamellar Body Protein antibody
ABC 3; ABC C; ABC C transporter; ABC transporter 3; ABC-C transporter; ABC3; ABCA 3; Abca3; ABCA3 protein; ABCA3_HUMAN; ABCC; ATP binding cassette 3; ATP binding cassette sub family A (ABC1) member 3; ATP binding cassette sub family A member
Cat:
SL17588R
Species Reactivity:
(predicted: Human,Mouse,Rat,Dog,Cow,Sheep,)
Immunogen:
KLH conjugated synthetic peptide derived from human ABCA3/P180 Lamellar Body Protein:65-160/1704
Format:
Liquid
Storage instructions:
Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Concentration:
1mg/ml
Clonality:
Polyclonal
Isotype:
IgG
Applications:
ELISA=1:5000-10000IHC-P=1:100-500IHC-F=1:100-500ICC=1:100-500IF=1:100-500(Paraffin sections need to do antigen repair)not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.
Host:
Rabbit
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Unit:
Price: $
Product PDFs
Datasheet:


The membrane-associated 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 intracellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). This protein is a member of the ABC1 subfamily. Members of the ABC1 subfamily comprise the only major ABC subfamily found exclusively in multicellular eukaryotes. The full transporter encoded by this gene may be involved in development of resistance to xenobiotics and engulfment during programmed cell death. [provided by RefSeq, Jul 2008]

Function:
Plays an important role in the formation of pulmonary surfactant, probably by transporting lipids such as cholesterol.

Subcellular Location:
Membrane.

Tissue Specificity:
Highly expressed in lung, followed by brain, pancreas, skeletal muscle and heart. Weakly expressed in placenta, kidney and liver. Also expressed in medullary thyroid carcinoma cells (MTC) and in SLCcell carcinoma.

DISEASE:
Defects in ABCA3 are the cause of pulmonary surfactant metabolism dysfunction type 3 (SMDP3) [MIM:610921]; also called pulmonary alveolar proteinosis due to ABCA3 deficiency. A rare lung disorder due to impaired surfactant homeostasis. It is characterized by alveolar filling with floccular material that stains positive using the periodic acid-Schiff method and is derived from surfactant phospholipids and protein components. Excessive lipoproteins accumulation in the alveoli results in severe respiratory distress.

Similarity:
Belongs to the ABC transporter superfamily. ABCA family.
Contains 2 ABC transporter domains.

SWISS:
Q99758

Gene ID:
21

Database links:

Entrez Gene: 21 Human

Entrez Gene: 27410 Mouse

Entrez Gene: 302973 Rat

Omim: 601615 Human

SwissProt: Q99758 Human

SwissProt: Q8R420 Mouse

Unigene: 26630 Human

Unigene: 239470 Mouse

Unigene: 233424 Rat



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