Close
Close
Loading...
CONTACT US
:
:
:
Call us at:
:
:
:
Fax:
Email:

ABCD1

Products

Loading...

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

Background

The ATP-binding cassette subfamily D member 1 (ABCD1) is a peroxisomal ATP-binding cassette half-transporter, with a single nucleotide-binding domain (NBD) and six transmembrane helices making up the substrate-translocation pathway, encoded by the ABCD1 gene on human chromosome Xq28. ABCD1 is best characterized for its role in peroxisomal import of very long-chain fatty acid (VLCFA)-CoA esters. Once inside the organelle, these fatty acids are degraded by β-oxidation, a catabolic mechanism that shortens the acyl chain and avoids toxic accumulation. Biochemical studies have shown that ABCD1 has intrinsic acyl-CoA thioesterase (ACOT) activity and releases VLCFA from acyl-CoA prior to or concomitant with membrane translocation as free fatty acid and CoA. This enzymatic activity is coupled to the transport process, and mutations that disrupt ACOT activity can also affect substrate translocation. Pathogenic mutations in ABCD1, including missense, nonsense, frameshift, and splice-site variants, can disrupt peroxisomal VLCFA metabolism and lead to the pathological lipid accumulation that characterizes X-linked adrenoleukodystrophy (X-ALD).

Fig.1 Therapeutic Targets for ABCD1 Mutations. (OA Literature)Fig.1 Therapeutic Targets in X-ALD Linked to ABCD1 Mutations.1

ABCD1 Protein Function: Peroxisomal VLCFA Transport and Lipid Metabolism

ABCD1 has been studied for its biological properties across multiple inter-related metabolic and disease contexts:

  • VLCFA Translocation and Detoxification: ABCD1 is the primary mechanism by which cells import VLCFAs into peroxisomes for degradation. When ALDP is not functional, plasma, brain white matter, adrenal cortex and testicular Leydig cells accumulate excessive amounts of the long-chain fatty acids C24:0 and C26:0 by orders of magnitude more than physiological concentrations. This accumulation represents the main biochemical feature of X-ALD and has therefore been used as an established biomarker for diagnosis.
  • Mitochondrial-Peroxisomal Crosstalk: Besides its direct transport function, ABCD1 seems to mediate a physical and metabolic interaction between peroxisomes and mitochondria. Although fatty acid β-oxidation in isolated peroxisomes from the Abcd1 knockout mouse is normal, abnormal accumulation of VLCFA occurs, suggesting that ALDP deficiency may disturb inter-organellar metabolite channeling. In accordance, mitochondrial structural abnormalities have been demonstrated in ALD mouse adrenal cortical cells indicating a general bioenergetic dysfunction within the context of cell pathogenesis of this disorder.
  • Disease Phenotype Spectrum: ABCD1 mutations display an incredibly heterogeneous clinical spectrum even within individual families. ccALD is associated with rapidly progressive demyelination with neuroinflammation when it occurs in childhood (ccALD) between the ages of 4 and 10 years. Adrenomyeloneuropathy (AMN) diagnosed in adulthood presents as a slowly progressive axonopathy of the spinal cord, associated with spastic paraparesis and bladder dysfunction.

ABCD1 Membrane Protein Product

Jumpstart your gene and metabolism programs with our well-characterized glycolipids catalog of recombinant ABCD1 membrane proteins. We acknowledge that the proper orientation in the peroxisomal membrane, homodimeric assembly and ATP-dependent conformational cycling of ABCD1 are significant challenges for production. To address these challenges, we utilize a variety of expression platforms to produce stable and correctly folded, membrane-embedded ABCD1 constructs that maintain native ATPase activity and substrate-binding characteristics. ALDP is offered in either full-length wild-type, or disease-associated forms mouse. Each preparation is subject to thorough biophysical validation by SDS-PAGE, analytical size-exclusion chromatography, ATPase activity assays and ACOT functional measurements to ensure appropriateness for structural studies, inhibitor screening and antibody development.

ABCD1 Protein Product

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

ABCD1 Stable Cell Line Product

Reliable and reproducible cellular models are needed to elucidate ABCD1-driven biology and test therapeutic candidates. We have generated stable cell lines expressing wild-type or pathogenic human, mouse and rat ABCD1, as well as knocked-down endogenous expression for loss-of-function studies. These platforms can be accurately optimized for VLCFA uptake assays, peroxisomal β-oxidation quantification, ATPase activity measurements as well as high-throughput screening of pharmacological chaperones or gene therapy vectors – providing the experimental reproducibility necessary to coordinate needs across multi-phase research initiatives.

ABCD1 Stable Cell Line Product

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

ABCD1 Recombinant Antibody Product

As a leader in the field with years of experience, our catalog offers high-affinity anti-ABCD1 recombinant monoclonal antibodies that hold immense potential for strict researchers. Produced with cutting-edge recombinant technologies, these antibodies provide improved specificity, sensitivity and batch-to-batch consistency compared to traditional polyclonal antibodies. Our recombinant ABCD1 antibodies are confirmed for WB, ELISA, FCM, IF, ICC, IHC and IP applications that deliver high specificity in both detection and quantification of ABCD1 protein in multiple sample types such as peroxisomal membrane preparations, human fibroblast lysates and X-ALD tissues.

ABCD1 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 ABCD1 Membrane Protein and Antibody Discovery Services

In addition to our catalog products, we provide customized services focused on membrane protein and antibody discovery and development. Drawing on our unique experience in both peroxisomal function as well as in rare disease, we can support you with:

  • Custom ABCD1 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. Are your ABCD1 products suitable for clinical or diagnostic use?

    No, these products/services are not intended for clinical diagnosis, prevention, treatment or cure of any disease.

  2. What species variants are available?

    We offer mouse and rat ABCD1 reagents in our catalog. However, additional orthologs or engineered mutants can be produced upon request.

  3. Do you handle bulk or custom formulations for high-throughput screening?

    Yes. We offer a tier of pricing plans flexible enough to meet both theoretical and practical level needs for academia and industry scale up requirements. Submit your expected volume and time in our inquiry portal to get a customized quote.

  4. Do you have stable cell lines with inducible ABCD1 expression or co-expressed peroxisomal markers?

    Absolutely. Our cell engineering team routinely generates tetracycline-inducible systems and lines co-expressing ABCD1 with PMP70, catalase, or peroxisomal targeting signal reporters for comprehensive organelle transport studies. We welcome detailed discussions regarding your preferred expression architecture and phenotypic requirements.

Reference
  1. Zuo, Xinxin, and Zeyu Chen. "From gene to therapy: a review of deciphering the role of ABCD1 in combating X-linked adrenoleukodystrophy." Lipids in health and disease 23.1 (2024): 369. Under Open Access license CC BY 4.0, without modification. https://doi.org/10.1186/s12944-024-02361-0
Our customer service representatives are available 24 hours a day, 7 days a week. Contact Us
© 2026 Creative Biolabs. | Contact Us
;