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ABCD3

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All listed services and products are For Research Use Only. Do Not use in any diagnostic or therapeutic applications.

Background

ATP-binding cassette sub-family D member 3 (ABCD3) is a half-size ATP-binding cassette (ABC) transporter with a molecular weight of approximately 70–75 kDa that is localized in the peroxisomal membrane. ABCD3 mediates the import of a wide array of CoA-esterified substrates from the cytosol into peroxisomes for β-oxidation and other catabolic processes. Its substrates include long-chain fatty acyl-CoAs, branched-chain fatty acids (e.g., phytanic acid), dicarboxylic acids, as well as the C27 bile acid intermediates dihydroxycholestanoic acid (DHCA) and trihydroxycholestanoic acid (THCA). ABCD3 also has intrinsic fatty acyl-CoA thioesterase activity that hydrolyzes fatty acyl-CoA substrates to free fatty acids and CoA before or during ATP-dependent peroxisomal transport. This enzymatic activity is coupled to the transport mechanism of ABCD3 and contributes to the translocation of its lipid substrates across the peroxisomal membrane.

Fig.1 Peroxisomal ABC transporters, including ABCD3. (OA Literature)Fig.1 Peroxisomal ABC transporters and their involvement in lipid metabolism.1

ABCD3 Protein Function: A Versatile Gatekeeper of Peroxisomal Lipid Metabolism

ABCD3 biological impact—a key and pleiotropic regulator of several metabolic and pathological processes-spans multiple overlapping domains:

  • Fatty Acid Oxidation and Energy Homeostasis: ABCD3 is critical for peroxisomal β-oxidation of various lipid classes. It facilitates the entry of long-chain, branched-chain and dicarboxylic fatty acyl-CoAs into the peroxisomal matrix to have them enzymatically shortened in acyl chains that cannot be handled by mitochondrial β-oxidation. Metabolic profiling in ABCD3 knockout models validated its predominance for hepatic dicarboxylic fatty acid metabolism and overall liver lipid homeostasis.
  • Bile Acid Biosynthesis: Transport of C27 bile acid precursors (DHCA-CoA and THCA-CoA) into peroxisomes is an important step in the biosynthesis pathway which converts cholesterol-derived intermediates to mature C24 bile acids. Deficiency of ABCD3 function abolishes this route, resulting in toxic bile acid intermediates and pronounced liver injury. This function illustrates the non-overlapping role of ABCD3 in enterohepatic trafficking and digestive physiology.
  • Disease Associations: Mutations in ABCD3 are linked to disease with congenital bile acid synthesis defect 5 (CBAS5), a rare condition that leads to profound liver disease and increased plasma bile acid intermediates. ABCD3 expression is altered in other metabolic conditions, including those which bear a resemblance to Zellweger syndrome spectrum disorders, such as intrahepatic cholestasis and oculopharyngodistal myopathy. In oncology, the heterogeneous function of ABCD3 has been proposed as a poor-prognostic marker in colorectal cancer due to reduced levels of mRNA but amplification has also been reported to confer chemoresistance in drug-resistant cancer cell lines, reflecting its context-dependent functions as a tumor suppressor and promoter of chemoresistance.

ABCD3 Membrane Protein Product

Accelerate your peroxisomal metabolism and lipid biology projects with our high quality recombinant ABCD3 membrane protein catalogue. The localization of both the peroxisomal membrane, as well as the homodimeric assembly and ATP-dependent conformational cycling of ABCD3 pose unique secretion concerns. We overcome these by utilizing a range of expression systems to obtain well-folded and functional, membrane-embedded ABCD3 constructs that maintain native ATPase and substrate-binding capabilities. Featuring complete-length mouse PMP70 as well as disease-associated variants. All preparations are biophysically characterized by functional measurements for being used in structural studies, inhibitor screening or antibody development.

ABCD3 Protein Product

Not finding the membrane protein product you need? Contact us to start your one-stop custom service!

ABCD3 Stable Cell Line Product

A comprehensive understanding of the ABCD3 driven biology and identification of key therapeutic candidates critically depend on reliable, reproducible cellular models. Our stable cell lines expressing wild-type or pathogenic forms of ABCD3 (mouse or rat) as well as anti-ABCD3 knockdown for loss-of-function studies are available. The platforms are further finely tuned for fatty acid oxidation assays, assessment of bile acid precursor transport, determination of lipid droplet-peroxisome contacts and high-throughput screening of pharmacological modulators to provide the level consistency needed across multiple phases of research.

ABCD3 Stable Cell Line Product

Not finding the stable cell line product you need? Contact us to start your one-stop custom service!

ABCD3 Recombinant Antibody Product

Our extensive collection includes high-affinity recombinant antibodies against ABCD3, designed to serve diverse research requirements. These engineered research reagents offer high specificity, sensitivity, and consistent lot-to-lot reproducibility. ABCD3 recombinant antibodies may be suitable or available for selected research applications, including Western Blotting (WB), ELISA, Flow Cytometry (FCM), Immunofluorescence (IF), Immunocytochemistry (ICC), Immunohistochemistry (IHC), and/or Immunoprecipitation (IP), depending on the specific product. They can support the detection of ABCD3 in various research sample types, such as peroxisomal membrane preparations, tissue lysates, and tissue sections from model systems.

ABCD3 Recombinant Antibody Product

Not finding the recombinant antibody product you need? Contact us to start your one-stop custom service!

Product Features

  • High Purity & Activity: High purity and verified integrity for dependable downstream work.
  • Diverse Expression Systems: Choice of expression formats to match routine or specialized projects.
  • Species Reactivity: Products available for human, mouse, pig, and other species.
  • Validated Applications: Application-tested to deliver consistent signals across common lab workflows.
  • Exceptional Lot-to-Lot Consistency: Tight lot-to-lot control for reproducible performance.
  • Customization Options: Flexible customization of tags, labels, or packaging upon request.

Custom ABCD3 Membrane Protein and Antibody Discovery Services

In addition to our catalog products, we provide specialized custom services for membrane protein and antibody discovery and development. We are always at your service based on our comprehensive knowledge in peroxisomal biology and metabolic diseases:

  • Custom ABCD3 Receptor Production: Tailored expression, purification, and characterization of challenging multi-domain receptor constructs.
  • Custom Antibody Development: From antigen design to antibody engineering (monoclonal, polyclonal, recombinant) for specific research applications.
  • Stable Cell Line Development: Generation of bespoke stable cell lines expressing your target of interest.
  • Functional Assay Development: Designing and executing assays to assess receptor activation and ligand.

Frequently Asked Questions (FAQ)

  1. Why is ABCD3 a key target of investigation?

    ABCD3 is a highly abundant peroxisomal membrane protein and acting as a key gatekeeper of peroxisomal lipid metabolism. Congenital bile acid synthesis defect 5 (CBAS5) results from mutations and is associated with disorders of the Zellweger syndrome spectrum. ABCD3 regulates lipid droplet-peroxisome contacts and chemoresistance in cancer. It is crucial to understand its transport mechanism for developing therapies against metabolic and neoplastic diseases.

  2. Are your ABCD3 products for clinical or diagnostic use?

    No, all ABCD3 products and services are for research use only; they are not to be used for clinical diagnosis, prevention, treatment or cure of any disease.

  3. What types of ABCD3 products do you offer?

    We offer a range of products including recombinant full-length ABCD3, disease-associated variants, stable cell lines with ABCD3 overexpression or knockdown, and high-quality recombinant ABCD3 antibodies, along with comprehensive custom services.

  4. Do you offer stable cell lines with inducible ABCD3 expression or co-expression with peroxisomal markers?

    Absolutely. The engineered cell lines are then raised that co-express ABCD3 with PMP70, catalase or other PEX proteins and lipid droplet markers to monitor organelle interactions on a routine basis by our cell engineering team. We are open to discussion of your preferred expression architecture and phenotypic needs in detail.

Reference
  1. Tawbeh, Ali, et al. "Peroxisomal ABC transporters: an update." International Journal of Molecular Sciences 22.11 (2021): 6093. Under Open Access license CC BY 4.0, without modification. https://doi.org/10.3390/ijms22116093
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