Mouse Anti-ABCA3 Recombinant Antibody (V2-178912) (CBMAB-A0146-YC)

Name: Mouse Anti-ABCA3 Recombinant Antibody (V2-178912)
SKU: CBMAB-A0146-YC
Rating: 5 (1 reviews)

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Datasheet

SDS

Request for COA

Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Mouse

Clone

V2-178912

Application

WB, ICC, IHC-P, IHC-Fr, ELISA

Immunogen

ATP Binding Cassette Transporter A3

Specificity

Human

Antibody Isotype

IgG

Clonality

Monoclonal

Application Notes

The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Application	Note
WB	1:500-1:5000
IHC	1:50-1:200
IF(ICC)	1:50-1:200

Formulations & Storage [For reference only, actual COA shall prevail!]

Format

PBS, pH 7.4, 0.02% sodium azide, 50% glycerol

Buffer

PBS, pH7.4, 50% Glycerol

Preservative

0.02% sodium azide

Concentration

1 mg/ml

Storage

Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

More Infomation

Target

Full Name

ATP Binding Cassette Subfamily A Member 3

Introduction

ABCA3 belongs to 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, W

Entrez Gene ID

UniProt ID

Q99758

Alternative Names

ATP Binding Cassette Subfamily A Member 3; ATP-Binding Cassette, Sub-Family A (ABC1), Member 3; ATP-Binding Cassette Transporter 3; ABC-C Transporter; ABC3; ATP-Binding Cassette Sub-Family A Member 3; ATP-Binding Cassette 3; ABC Transporter 3;

Function

Catalyzes the ATP-dependent transport of phospholipids such as phosphatidylcholine and phosphoglycerol from the cytoplasm into the lumen side of lamellar bodies, in turn participates in the lamellar bodies biogenesis and homeostasis of pulmonary surfactant. Transports preferentially phosphatidylcholine containing short acyl chains. In addition plays a role as an efflux transporter of miltefosine across macrophage membranes and free cholesterol (FC) through intralumenal vesicles by removing FC from the cell as a component of surfactant and protects cells from free cholesterol toxicity.

Biological Process

Cellular protein metabolic process
Drug export
Drug transmembrane transport
Lipid transport
Lung development
Organelle assembly
Phosphatidylcholine metabolic process
Phosphatidylglycerol metabolic process
Phospholipid homeostasis
Phospholipid transport
Positive regulation of cholesterol efflux
Positive regulation of phospholipid efflux
Positive regulation of phospholipid transport
Positive regulation of protein homooligomerization
Regulation of lipid biosynthetic process
Regulation of phosphatidylcholine metabolic process
Response to drug
Response to glucocorticoid
Surfactant homeostasis
Xenobiotic transport

Cellular Location

Multivesicular body membrane; Late endosome membrane; Lysosome membrane; Cytoplasmic vesicle membrane. Localized in the limiting membrane of lamellar bodies in lung alveolar type II cells. Trafficks via the Golgi, sorting vesicles (SVs) and late endosome/multivesicular body network directly to the outer membrane of lamellar bodies in AT2 lung epithelial cells or to lysosomes and lysosomal-related organelles (LROs) in other cells where undergoes proteolytic cleveage and oligosaccharide processing from high mannose type to complex type. Oligomers formation takes place in a post-endoplasmic reticulum compartment.

Involvement in disease

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.

PTM

N-glycosylated. Localization at intracellular vesicles is accompanied by processing of oligosaccharide from high mannose type to complex type. N-linked glycosylation at Asn-124 and Asn-140 is required for stability and efficient anterograde trafficking and prevents from proteasomal degradation.
Proteolytically cleaved by CTSL and to a lower extent by CTSB within multivesicular bodies (MVB) and lamellar bodies (LB) leading to a mature form of 150 kDa.

Wambach, J. A., Yang, P., Wegner, D. J., Heins, H. B., Luke, C., Li, F., ... & Cole, F. S. (2020). Functional genomics of ABCA3 variants. American Journal of Respiratory Cell and Molecular Biology, 63(4), 436-443.

Shaw, N., Kicic, A., Schneider-Daum, N., Fletcher, S., Wilton, S., Huwer, H., ... & Schultz, A. (2020). Characterisation of a surrogate Type-II alveolar cell culture model for ATP binding cassette subfamily a member 3 (ABCA-3) deficiency. Respirology, 25(S1), 130.

Hu, J. Y., Yang, P., Wegner, D. J., Heins, H. B., Luke, C. J., Li, F., ... & Wambach, J. A. (2020). Functional characterization of four ATP‐binding cassette transporter A3 gene (ABCA3) variants. Human mutation, 41(7), 1298-1307.

Li, Y., Kinting, S., Höppner, S., Forstner, M. E., Uhl, O., Koletzko, B., & Griese, M. (2019). Metabolic labelling of choline phospholipids probes ABCA3 transport in lamellar bodies. Biochimica et Biophysica Acta (BBA)-Molecular and Cell Biology of Lipids, 1864(12), 158516.

Denman, L., Yonker, L. M., & Kinane, T. B. (2018). The classification of ATP‐binding cassette subfamily A member 3 mutations using the cystic fibrosis transmembrane conductance regulator classification system. Pediatric investigation, 2(1), 17-24.

Schindlbeck, U., Wittmann, T., Höppner, S., Kinting, S., Liebisch, G., Hegermann, J., & Griese, M. (2018). ABCA3 missense mutations causing surfactant dysfunction disorders have distinct cellular phenotypes. Human mutation, 39(6), 841-850.

Kinane, T. B., Denman, L., & Yonker, L. (2017). The Classification Of Atp-Binding Cassette Subfamily A Member 3 (abca3) Mutations Using The Cystic Fibrosis Transmembrane Conductance Regulator (cftr) Classification System. In C77. ADVANCEMENTS IN THE BIOLOGY OF BPD AND OTHER CONGENITAL PEDIATRIC LUNG DISEASES (pp. A6405-A6405). American Thoracic Society.

Kröner, C., Wittmann, T., Reu, S., Teusch, V., Klemme, M., Rauch, D., ... & Griese, M. (2017). Lung disease caused by ABCA3 mutations. Thorax, 72(3), 213-220.

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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

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For research use only. Not intended for any clinical use.

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