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ABCD1 (show ABCD1 ELISA Kits) and ABCD2 (show Abcd2 ELISA Kits) are involved in the transport of long and very long chain fatty acids (VLCFA) or their CoA-derivatives into peroxisomes with different substrate specificities, while ABCD3 is involved in the transport of branched chain acyl-CoA (show GNPAT ELISA Kits) into peroxisomes.ABCD4 is deduced to take part in the transport of vitamin B12 (show NDUFB3 ELISA Kits) from lysosomes into the cytosol.
Data show that ACBD3 (show Acbd3 ELISA Kits) can recruit PI4KB (show PI4KB ELISA Kits) to model membranes as well as redirect PI4KB (show PI4KB ELISA Kits) to cellular membranes where it is not naturally found. Also, results show that ACBD3 (show Acbd3 ELISA Kits) regulates the enzymatic activity of PI4KB (show PI4KB ELISA Kits) kinase through membrane recruitment rather than allostery.
The N-terminal motif of PMP70 suppresses cotranslational targeting to the endoplasmic reticulum.
Increased ABCD3 expression correlates with Gleason Score.
Deficiency of peroxisomal ABCD3 resulted in bile acid biosynthesis defect.
We postulate a role for human ABCD3 in the oxidation of dicarboxylic acids and a role in buffering fatty acids that are overflowing from the mitochondrial beta-oxidation system
investigation of organelle-targeting properties of N-terminal portions of peroxisomal PMP70; amino acid sequence and domain structure of human form discussed
Pex19p binds to PMP70 co-translationally and keeps PMP70 in a proper conformation for the localization to peroxisome.
Testosterone metabolites did not alter expression of ABCD3 mRNA in fibroblasts from X-linked adrenoleukodystrophy patients.
MP70 (show GJA8 ELISA Kits) interacts with PEX19 (show PEX19 ELISA Kits) splice variants PEX19 (show PEX19 ELISA Kits)-delta-E2 and PEX19p-delta-E8.
Abcd3-/- mice accumulated the branched chain fatty acid phytanic acid after phytol loading.
investigation of organelle-targeting properties of N-terminal portions of peroxisomal PMP70; mouse full-length sequence & deletion sequences used as probes for intracellular targeting in COS cells
reduction of PMP70 might underlie decrease in peroxisomal functions and increase in oxidative stress in amyotrophic lateral sclerosis
PPARalpha (show PPARA ELISA Kits) up-regulates the expression of Abcd3 in small intestine.
The protein encoded by this gene 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, GCN20, White). This protein is a member of the ALD subfamily, which is involved in peroxisomal import of fatty acids and/or fatty acyl-CoAs in the organelle. All known peroxisomal ABC transporters are half transporters which require a partner half transporter molecule to form a functional homodimeric or heterodimeric transporter. This peroxisomal membrane protein likely plays an important role in peroxisome biogenesis. Mutations have been associated with some forms of Zellweger syndrome, a heterogeneous group of peroxisome assembly disorders. Alternative splicing results in multiple transcript variants encoding distinct isoforms.
70 kDa peroxisomal membrane protein
, ATP-binding cassette sub-family D member 3
, Peroxisomal membrane protein-1 (70kD)
, dJ824O18.1 (ATP-binding cassette, sub-family D (ALD), member 3 (PMP70, PXMP1))
, peroxisomal membrane protein 1 (70kD, Zellweger syndrome)
, 68 kDa peroxisomal membrane protein
, peroxisomal membrane protein 1
, peroxisomal membrane protein, 70 kDa
, ATP-binding cassette, sub-family D, member 3
, ATP-binding cassette, sub-family D (ALD), member 3
, 70-kDa peroxisomal membrane protein
, Peroxisomal membrane protein 1
, ATP-binding cassette, sub-family D, member 3-like
, ATP-binding cassette sub-family D member 3-like