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Rabbit Anti-ABCD1 Recombinant Antibody (V2-179060) (CBMAB-A0296-YC)

Provided herein is a Rabbit monoclonal antibody against Human ATP Binding Cassette Subfamily D Member 1. The antibody can be used for immunoassay techniques, such as WB, ICC, IF.
See all ABCD1 antibodies
Published Data
Rabbit Anti-ABCD1 Recombinant Antibody (CBYC-A148) (CBMAB-A0296-YC) in WB
Representative Western blot images show rAAV9-CBA-hABCD1 expression in various organs after intravenous (IV) injection in spinal cord. ...View More
Gong, Y., Berenson, A., Laheji, F., Gao, G., Wang, D., Ng, C., ... & Eichler, F. (2019). Intrathecal adeno-associated viral vector-mediated gene delivery for adrenomyeloneuropathy. Human Gene Therapy, 30(5), 544-555.
Rabbit Anti-ABCD1 Recombinant Antibody (CBYC-A148) (CBMAB-A0296-YC) in IF
Representative images show rAAV9-CBA-hABCD1-HA distribution across the whole spinal cord after IT pump delivery at 2 × 1011 GC/mouse. Higher magnification image shows punctate HA tag expression (HA, green) in glial cells and some neurons (NF, red). ...View More
Gong, Y., Berenson, A., Laheji, F., Gao, G., Wang, D., Ng, C., ... & Eichler, F. (2019). Intrathecal adeno-associated viral vector-mediated gene delivery for adrenomyeloneuropathy. Human Gene Therapy, 30(5), 544-555.

Summary

Host Animal
Rabbit
Specificity
Human, Mouse, Rat
Clone
V2-179060
Antibody Isotype
IgG
Application
WB, ICC, IF

Basic Information

Immunogen
Recombinant fragment.
Specificity
Human, Mouse, Rat
Antibody Isotype
IgG
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.
ApplicationNote
WB1:10,000
IF(ICC)1:100

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

Buffer
PBS, 40% Glycerol, 0.05% BSA
Preservative
0.01% sodium azide
Concentration
Batch dependent
Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
ATP Binding Cassette Subfamily D Member 1
Introduction
ABCD1 is a member of the superfamily of ATP-binding cassette (ABC) transporters. ABC proteins transport various molecules across extra- and intra-cellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN
Entrez Gene ID
Human215
Mouse11666
Rat363516
UniProt ID
HumanP33897
MouseP48410
RatD3ZHR2
Alternative Names
ATP Binding Cassette Subfamily D Member 1; ATP-Binding Cassette, Sub-Family D (ALD), Member 1; Adrenoleukodystrophy Protein; ALDP; ALD; ATP-Binding Cassette Sub-Family D Member 1; ABC42; AMN;
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.
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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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