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Protein Page:
ABCD1 (human)
p Phosphorylation
ac Acetylation
me Methylation
m1 Mono-methylation
m2 Di-methylation
m3 Tri-methylation
ub Ubiquitylation
sm Sumoylation
ne Neddylation
gl O-GlcNAc
ga O-GalNAc
pa Palmitoylation
ad Adenylation
sn S-Nitrosylation
ca Caspase cleavage
sc Succinylation

Overview
ABCD1 Probable transporter. The nucleotide-binding fold acts as an ATP-binding subunit with ATPase activity. Defects in ABCD1 are the cause of adrenoleukodystrophy X- linked (X-ALD). X-ALD is 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. The promoter region of ABCD1 is deleted in the chromosome Xq28 deletion syndrome which involves ABCD1 and the neighboring gene BCAP31. Belongs to the ABC transporter superfamily. ABCD family. Peroxisomal fatty acyl CoA transporter (TC 3.A.1.203) subfamily. Note: This description may include information from UniProtKB.
Protein type: Mitochondrial; Hydrolase; Membrane protein, multi-pass; Transporter; Membrane protein, integral; Transporter, ABC family
Chromosomal Location of Human Ortholog: Xq28
Cellular Component: cytoplasm; cytosol; integral to peroxisomal membrane; membrane; perinuclear region of cytoplasm; peroxisomal membrane; peroxisome
Molecular Function: ATP binding; ATPase activity; enzyme binding; identical protein binding; long-chain fatty acid transporter activity; peroxisomal fatty acyl CoA transporter activity; protein binding; protein homodimerization activity
Biological Process: fatty acid beta-oxidation; fatty acid beta-oxidation using acyl-CoA oxidase; linoleic acid metabolic process; long-chain fatty acid catabolic process; peroxisomal long-chain fatty acid import; peroxisome organization and biogenesis; transmembrane transport; very-long-chain fatty acid catabolic process
Disease: Adrenoleukodystrophy
Reference #:  P33897 (UniProtKB)
Alt. Names/Synonyms: ABC42; ABCD1; Adrenoleukodystrophy protein; ALD; ALDP; AMN; ATP-binding cassette sub-family D member 1; ATP-binding cassette, sub-family D (ALD), member 1
Gene Symbols: ABCD1
Molecular weight: 82,937 Da
Basal Isoelectric point: 9.09  Predict pI for various phosphorylation states
Select Structure to View Below

ABCD1

Protein Structure Not Found.


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Modification Sites and Domains Show Modification Legend
Click here to view phosphorylation modifications only

Modification Sites in Parent Protein, Orthologs, and Isoforms Show Modification Legend
 

Show Multiple Sequence Alignment



 LTP 

LTP: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.


 HTP 

HTP: The number of records in which this modification site was assigned using ONLY proteomic discovery-mode mass spectrometry.


       human

 
0 1 T18 RGNTLKRTAVLLALA
0 1 S58‑p GEPTQEAsGVAAAKA
0 1 Y180 LYFSQQTYYRVSNMD
0 1 Y181 YFSQQTYYRVSNMDG
0 1 Y337‑p LEQFLMKyVWSASGL
0 1 Y357‑p PIITATGysEsDAEA
0 1 S358‑p IITATGysEsDAEAV
0 1 S360‑p TATGysEsDAEAVKK
0 1 K407‑ub ERIMSSYkEVTELAG
0 2 S453‑p GSGTIGRsGVRVEGP
0 1 K602 WEAMCDWKDVLSGGE
0 1 K696 RLSLTEEKQRLEQQL
0 1 K708‑ub QQLAGIPkMQRRLQE
0 6 S733‑p PAHVPAPsPQGPGGL
  mouse

 
T18‑p RVTTLKRtAVVLALT
S58 GEPTQEASGATATKA
Y180‑p LYFSQQTyyRVSNMD
Y181‑p YFSQQTyyRVSNMDG
Y337 LEQFLMKYVWSASGL
Y357 PIITATGYAESDSEA
A358 IITATGYAESDSEAM
S360 TATGYAESDSEAMKK
K407 ERIMSSYKEVTELAG
S453‑p GPGVMVQsGVHVEGP
K602‑ub WEAVCDWkDVLSGGE
K696‑ub RLSLTEEkQRLEQQL
K708 QQLAGIPKMQGRLQE
- gap
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