Mouse Abcd1 ELISA Kit (V2LY-0626-LY1856)

Name: Mouse Abcd1 ELISA Kit
SKU: V2LY-0626-LY1856
Rating: 5 (1 reviews)

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Tested Data

Datasheet

SDS

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

Basic Information

Sensitivity

0.0075 ng/mL

Detection Range

0.015-3 ng/mL

Sample Type

Serum, Plasma, cell culture supernates

Specificity

Mouse

Assay Type

Sandwich

Reactivity

Mouse

Assay Time

1.5 h

Molecule Mass

81.9 kDa

Components

Pre-coated ELISA Plate: 12 wells * 8 detachable strips
Standard solution: 0.5ml x1
Standard diluent: 3ml x1
Streptavidin-HRP: 6ml x1
Stop solution: 6ml x1
Substrate solution A: 6ml x1
Substrate solution B: 6ml x1
Wash buffer concentrate (25x): 20ml x1
Biotinylated antibody: 1ml x1

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

Storage

Store at 2-8°C

More Infomation

Target

Full Name

ATP Binding Cassette Subfamily D Member 1

Function

Plays a role in the transport of free very-long-chain fatty acids (VLCFAs) as well as their CoA-esters across the peroxisomal membrane by acting as an ATP-specific binding subunit releasing ADP after ATP hydrolysis. Thus, plays a role in regulation of VLCFAs and energy metabolism namely, in the degradation and biosynthesis of fatty acids by beta-oxidation, mitochondrial function and microsomal fatty acid elongation. Involved in several processes; namely, controls the active myelination phase by negatively regulating the microsomal fatty acid elongation activity and may also play a role in axon and myelin maintenance. Controls also the cellular response to oxidative stress by regulating mitochondrial function like, mitochondrial oxidative phosphorylation and depolarization. And finally controls the inflammatory response by positively regulating peroxisomal beta-oxidation of VLCFAs (By similarity).

Biological Process

Alpha-linolenic acid metabolic process
Fatty acid beta-oxidation
Fatty acid beta-oxidation using acyl-CoA oxidase
Fatty acid elongation
Fatty acid homeostasis
Linoleic acid metabolic process
Lipid transport
Long-chain fatty acid catabolic process
Long-chain fatty acid import into peroxisome
Myelin maintenance Source: UniProtKB
Negative regulation of cytokine production involved in inflammatory response
Negative regulation of reactive oxygen species biosynthetic process
Neuron projection maintenance
Peroxisomal membrane transport
Peroxisome organization
Positive regulation of fatty acid beta-oxidation
Positive regulation of unsaturated fatty acid biosynthetic process
Protein import into peroxisome membrane
Regulation of cellular response to oxidative stress
Regulation of fatty acid beta-oxidation
Regulation of mitochondrial depolarization
Regulation of oxidative phosphorylation
Sterol homeostasis
Very long-chain fatty acid catabolic process
Very long-chain fatty-acyl-CoA catabolic process

Cellular Location

Lysosome membrane; Mitochondrion membrane; Endoplasmic reticulum membrane; Peroxisome membrane

Involvement in disease

A peroxisomal metabolic disorder characterized by progressive multifocal demyelination of the central nervous system and by peripheral adrenal insufficiency (Addison disease). It results in mental deterioration, corticospinal tract dysfunction, and cortical blindness. Different clinical manifestations exist like: cerebral childhood ALD (CALD), adult cerebral ALD (ACALD), adrenomyeloneuropathy (AMN) and 'Addison disease only' (ADO) phenotype.

PTM

Tyrosine-phosphorylated.

Hong, W., Yun, W., Choi, W., Son, D., Song, G., & You, S. (2021). Generation of a WA14 hESC sub-line carrying a hemizygous ABCD1 (C. 1696_1710 del) mutation introduced by CRISPR/Cas9 technology. Stem Cell Research, 52, 102244.

Cho, Y. K., Lee, S. Y., & Kim, S. W. (2020). Novel ABCD1 Gene Mutation in a Korean Patient with X-Linked Adrenoleukodystrophy Presenting with Addison's Disease. Endocrinology and Metabolism, 35(1), 188-191.

Richter Jr, J. E., Vadlamudi, C., Macklin, S. K., Samreen, A., Helmi, H., Broderick, D., ... & Caulfield, T. R. (2020). Characterization of a Pathogenic Variant in the ABCD1 Gene Through Protein Molecular Modeling. Case reports in genetics, 2020.

Schirinzi, T., Vasco, G., Aiello, C., Rizzo, C., Sancesario, A., Romano, A., ... & Cappa, M. (2019). Natural history of a cohort of ABCD 1 variant female carriers. European journal of neurology, 26(2), 326-332.

Wang, J., Zhu, Q., & Liu, H. (2018). A novel mutation in the ABCD1 gene of a Chinese patient with X-linked adrenoleukodystrophy: Case report. Medicine, 97(21).

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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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Isotype control

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

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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