Gene Ontology annotation through association of InterPro records with GO terms
Manual transfer of experimentally-verified manual GO annotation data to orthologs by curator judgment of sequence similarity
Annotation inferences using phylogenetic trees
Gene Ontology annotation based on UniProtKB/Swiss-Prot Subcellular Location vocabulary mapping, accompanied by conservative changes to GO terms applied by UniProt
Automatic transfer of experimentally verified manual GO annotation data to orthologs using Ensembl Compara
Automatic assignment of GO terms using logical inference, based on on inter-ontology links
Automatic Gene Ontology annotation based on Rhea mapping
Electronic Gene Ontology annotations created by ARBA machine learning models
Combined Automated Annotation using Multiple IEA Methods
Homo- and heterodimerization of peroxisomal ATP-binding cassette half-transporters.
Intraperoxisomal localization of very-long-chain fatty acyl-CoA synthetase: implication in X-adrenoleukodystrophy.
Human adrenoleukodystrophy protein and related peroxisomal ABC transporters interact with the peroxisomal assembly protein PEX19p.
Characterization and functional analysis of the nucleotide binding fold in human peroxisomal ATP binding cassette transporters.
Peroxisomal straight-chain Acyl-CoA oxidase and D-bifunctional protein are essential for the retroconversion step in docosahexaenoic acid synthesis.
Two splice variants of human PEX19 exhibit distinct functions in peroxisomal assembly.
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PEX19 variants bind ABCD1 as a membrane-protein client.
"Both interact with peroxisomal ABC transporters (ALDP, ALDRP, PMP70)"
Targeting of the human adrenoleukodystrophy protein to the peroxisomal membrane by an internal region containing a highly conserved motif.
Probing substrate-induced conformational alterations in adrenoleukodystrophy protein by proteolysis.
Molecular organization of peroxisomal enzymes: protein-protein interactions in the membrane and in the matrix.
ATP-binding and -hydrolysis activities of ALDP (ABCD1) and ALDRP (ABCD2), human peroxisomal ABC proteins, overexpressed in Sf21 cells.
Adrenoleukodystrophy: subcellular localization and degradation of adrenoleukodystrophy protein (ALDP/ABCD1) with naturally occurring missense mutations.
Live cell FRET microscopy: homo- and heterodimerization of two human peroxisomal ABC transporters, the adrenoleukodystrophy protein (ALDP, ABCD1) and PMP70 (ABCD3).
Distribution and cellular localization of adrenoleukodystrophy protein in human tissues: implications for X-linked adrenoleukodystrophy.
The human peroxisomal ABC half transporter ALDP functions as a homodimer and accepts acyl-CoA esters.
Defining the membrane proteome of NK cells.
The peroxisomal receptor Pex19p forms a helical mPTS recognition domain.
Peroxisomal localization of the proopiomelanocortin-derived peptides beta-lipotropin and beta-endorphin.
Differential substrate specificities of human ABCD1 and ABCD2 in peroxisomal fatty acid β-oxidation.
Very long chain fatty acid β-oxidation in astrocytes: contribution of the ABCD1-dependent and -independent pathways.
Impaired very long-chain acyl-CoA β-oxidation in human X-linked adrenoleukodystrophy fibroblasts is a direct consequence of ABCD1 transporter dysfunction.
Characterization of human ATP-binding cassette protein subfamily D reconstituted into proteoliposomes.
Acyl-CoA thioesterase activity of peroxisomal ABC protein ABCD1 is required for the transport of very long-chain acyl-CoA into peroxisomes.
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Reconstituted human ABCD1 preparations show ATPase and ACOT activity, with mechanistic and substrate-analogue limitations.
"The expressed ABCD1 possessed both ATPase and ACOT activities."
Putative X-linked adrenoleukodystrophy gene shares unexpected homology with ABC transporters.
Suppression of peroxisomal membrane protein defects by peroxisomal ATP binding cassette (ABC) proteins.
Translocation of tetracosahexaenoyl-CoA to peroxisomes
Translocation of tetracosapentaenoyl-CoA to peroxisomes
Linoleic acid (LA) metabolism
alpha-linolenic acid (ALA) metabolism
ABCD1-3 dimers transfer LCFAs from cytosol to peroxisomal matrix
PEX-19 docks ABCD1/D2/D3 to peroximal membrane
Peroxisomal uptake of very long-chain fatty acyl CoA
Defective ABCD1 does not transfer LCFAs from cytosol to peroxisomal matrix
PEX3:PEX19:class I PMP dissociates
PEX19:class I PMP binds PEX3
PEX19 binds class I peroxisomal membrane proteins
Impaired mitochondrial oxidative phosphorylation in the peroxisomal disease X-linked adrenoleukodystrophy.
Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage.
Brain microsomal fatty acid elongation is increased in abcd1-deficient mouse during active myelination phase.
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Mouse Abcd1 loss increases microsomal elongation during active myelination; this perturbation does not by itself establish an ABCD1-catalyzed elongation step.
"microsomal fatty acid elongation activity is stimulated in abcd1-deficient mice"
Abcd2 is a strong modifier of the metabolic impairments in peritoneal macrophages of ABCD1-deficient mice.
Silencing of Abcd1 and Abcd2 genes sensitizes astrocytes for inflammation: implication for X-adrenoleukodystrophy.
A key role for the peroxisomal ABCD2 transporter in fatty acid homeostasis.
Late onset neurological phenotype of the X-ALD gene inactivation in mice: a mouse model for adrenomyeloneuropathy.
Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy.
Astrocytes and mitochondria from adrenoleukodystrophy protein (ABCD1)-deficient mice reveal that the adrenoleukodystrophy-associated very long-chain fatty acids target several cellular energy-dependent functions.
X-linked adrenoleukodystrophy mice demonstrate abnormalities in cholesterol metabolism.
Early oxidative damage underlying neurodegeneration in X-adrenoleukodystrophy.
Evidence of oxidative stress in very long chain fatty acid--treated oligodendrocytes and potentialization of ROS production using RNA interference-directed knockdown of ABCD1 and ACOX1 peroxisomal proteins.
Structures of the human peroxisomal fatty acid transporter ABCD1 in a lipid environment.
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Human ABCD1 structures and ATPase modulation do not settle whether CoA is cleaved during translocation.
"It is currently unclear whether the fatty acyl chain is separated from its CoA ester for subsequent re-esterification"
Structural basis of substrate recognition and translocation by human very long-chain fatty acid transporter ABCD1.
ABCD1 functional-annotation research report (Falcon)
Structural insights into substrate recognition and translocation of human peroxisomal ABC transporter ALDP.
Structural and functional insights of the human peroxisomal ABC transporter ALDP.
Peroxisomal defects in microglial cells induce a disease-associated microglial signature.
Imbalanced mitochondrial dynamics contributes to the pathogenesis of X-linked adrenoleukodystrophy.
Pathophysiology of X-Linked Adrenoleukodystrophy: Updates on Molecular Mechanisms.
Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy.
The peroxisomal ABC transporter family.
From gene to therapy: a review of deciphering the role of ABCD1 in combating X-Linked adrenoleukodystrophy.
Gene and Cellular Therapies for Leukodystrophies.
Current Advances and Challenges in Gene Therapies for Neurologic Disorders: A Review for the Clinician.
An AAV-Based Therapy Approach for Neurological Phenotypes of X-Linked Adrenoleukodystrophy.