Datasheet

Biological Description

Specificity	ATP-binding cassette sub-family A member 3 Mouse mAb detects endogenous levels of total ATP-binding cassette sub-family A member 3 protein.
Background	ATP-binding cassette sub-family A member 3 (ABCA3) is a highly conserved, multi-pass transmembrane glycoprotein in the ABC transporter superfamily that uses ATP hydrolysis to transport phospholipids into lamellar bodies (LBs) of alveolar type II cells, where pulmonary surfactant is assembled and stored. Structurally, ABCA3 consists of two similar halves, each containing six transmembrane domains and a cytosolic nucleotide-binding domain (NBD) with Walker A, Walker B, and ABCA-specific signature motifs, plus two essential N-linked glycosylation sites at Asn124 and Asn140 required for proper folding, stability, and LB targeting. Although expressed in multiple tissues, ABCA3 is highly enriched in the lung, where it is critical for LB biogenesis and surfactant homeostasis, particularly in transporting phosphatidylcholine, phosphatidylglycerol, and cholesterol. Mutations in ABCA3 can cause trafficking or functional defects, leading to neonatal surfactant deficiency, interstitial lung disease, and fatal respiratory distress.

Specificity

ATP-binding cassette sub-family A member 3 Mouse mAb detects endogenous levels of total ATP-binding cassette sub-family A member 3 protein.

Background

ATP-binding cassette sub-family A member 3 (ABCA3) is a highly conserved, multi-pass transmembrane glycoprotein in the ABC transporter superfamily that uses ATP hydrolysis to transport phospholipids into lamellar bodies (LBs) of alveolar type II cells, where pulmonary surfactant is assembled and stored. Structurally, ABCA3 consists of two similar halves, each containing six transmembrane domains and a cytosolic nucleotide-binding domain (NBD) with Walker A, Walker B, and ABCA-specific signature motifs, plus two essential N-linked glycosylation sites at Asn124 and Asn140 required for proper folding, stability, and LB targeting. Although expressed in multiple tissues, ABCA3 is highly enriched in the lung, where it is critical for LB biogenesis and surfactant homeostasis, particularly in transporting phosphatidylcholine, phosphatidylglycerol, and cholesterol. Mutations in ABCA3 can cause trafficking or functional defects, leading to neonatal surfactant deficiency, interstitial lung disease, and fatal respiratory distress.

Usage Information

Application

IHC

Dilution

IHC

1:10

Reactivity

Mouse, Rat

Source

Mouse

MW

Storage Buffer

PBS, pH 7.2+50% Glycerol+0.05% BSA+0.01% NaN3

Storage
(from the date of receipt)

-20°C (avoid freeze-thaw cycles), 2 years

Exprimental Methods

References

https://pubmed.ncbi.nlm.nih.gov/28025703/

ATP-binding cassette sub-family A member 3 Mouse mAb

Biological Description

Usage Information

References

Application Data