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Identification
HMDB Protein ID HMDBP00102
Secondary Accession Numbers
  • 5334
Name Long-chain specific acyl-CoA dehydrogenase, mitochondrial
Synonyms
  1. LCAD
Gene Name ACADL
Protein Type Unknown
Biological Properties
General Function Involved in acyl-CoA dehydrogenase activity
Specific Function Not Available
Pathways
  • Carnitine palmitoyl transferase deficiency (I)
  • Carnitine palmitoyl transferase deficiency (II)
  • Ethylmalonic Encephalopathy
  • Fatty acid Metabolism
  • fatty acid metabolism
  • Glutaric Aciduria Type I
  • Long chain acyl-CoA dehydrogenase deficiency (LCAD)
  • Medium chain acyl-coa dehydrogenase deficiency (MCAD)
  • Mitochondrial Beta-Oxidation of Long Chain Saturated Fatty Acids
  • Mitochondrial Beta-Oxidation of Short Chain Saturated Fatty Acids
  • mitochondrial fatty acid beta-oxidation
  • PPAR signaling pathway
  • Short Chain Acyl CoA Dehydrogenase Deficiency (SCAD Deficiency)
  • Short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (SCHAD)
  • Trifunctional protein deficiency
  • Very-long-chain acyl coa dehydrogenase deficiency (VLCAD)
Reactions
Long-chain-acyl-CoA + electron-transfer flavoprotein → long-chain-2,3-dehydroacyl-CoA + reduced electron-transfer flavoprotein details
hexadecanoyl-CoA + FAD → (2E)-Hexadecenoyl-CoA + FADH details
Octanoyl-CoA + FAD → (2E)-Octenoyl-CoA + FADH details
Lauroyl-CoA + FAD → (2E)-Dodecenoyl-CoA + FADH details
Tetradecanoyl-CoA + FAD → (2E)-Tetradecenoyl-CoA + FADH details
Hexanoyl-CoA + FAD → trans-2-Hexenoyl-CoA + FADH details
Decanoyl-CoA (n-C10:0CoA) + FAD → (2E)-Decenoyl-CoA + FADH details
GO Classification
Biological Process
protein homotetramerization
carnitine catabolic process
carnitine metabolic process, CoA-linked
fatty acid beta-oxidation using acyl-CoA dehydrogenase
long-chain fatty acid catabolic process
negative regulation of fatty acid biosynthetic process
negative regulation of fatty acid oxidation
regulation of cholesterol metabolic process
temperature homeostasis
Cellular Component
mitochondrial membrane
mitochondrial matrix
Function
oxidoreductase activity, acting on the ch-ch group of donors
acyl-coa dehydrogenase activity
binding
catalytic activity
nucleoside binding
purine nucleoside binding
adenyl nucleotide binding
oxidoreductase activity
fad or fadh2 binding
Molecular Function
flavin adenine dinucleotide binding
fatty-acyl-CoA binding
long-chain-acyl-CoA dehydrogenase activity
palmitoyl-CoA oxidase activity
Process
metabolic process
oxidation reduction
Cellular Location
  1. Mitochondrion matrix
Gene Properties
Chromosome Location 2
Locus 2q34
SNPs ACADL
Gene Sequence
>1293 bp
ATGGCCGCACGCCTTCTCCGAGGGTCCCTACGCGTCCTGGGCGGCCACCGTGCGCCGCGC
CAGCTGCCCGCCGCGCGATGTTCTCATTCCGGAGGGGAAGAACGTCTAGAAACTCCTTCT
GCTAAAAAATTAACAGATATAGGAATTCGAAGAATCTTTTCTCCAGAGCATGACATTTTC
CGGAAAAGTGTAAGGAAGTTTTTCCAAGAAGAAGTGATTCCTCATCACTCAGAATGGGAG
AAAGCTGGAGAAGTAAGTAGGGAGGTTTGGGAAAAAGCTGGAAAACAAGGACTGCTTGGT
GTCAATATTGCAGAGCATCTTGGTGGAATTGGAGGGGATCTGTACTCCGCAGCTATTGTC
TGGGAGGAGCAAGCTTATTCAAATTGTTCAGGCCCAGGTTTTAGTATTCATTCAGGTATT
GTCATGTCCTATATTACAAACCATGGCTCAGAAGAACAGATTAAGCACTTTATTCCCCAG
ATGACTGCAGGCAAATGTATTGGTGCAATAGCAATGACAGAGCCTGGAGCTGGAAGTGAC
TTACAGGGAATAAAAACAAATGCTAAAAAGGATGGAAGTGACTGGATTCTCAATGGAAGC
AAGGTGTTCATCAGTAATGGGTCATTAAGTGATGTTGTGATTGTAGTTGCGGTCACAAAT
CATGAAGCTCCCTCCCCTGCCCATGGTATTAGCCTTTTTCTGGTGGAAAATGGAATGAAA
GGATTTATCAAGGGACGAAAGCTACATAAAATGGGATTAAAAGCCCAGGATACCGCAGAA
CTATTCTTTGAAGATATACGGTTGCCAGCTAGTGCCCTACTTGGAGAAGAGAATAAAGGC
TTCTATTACATCATGAAAGAGCTTCCACAGGAAAGGCTGTTAATTGCTGATGTGGCAATT
TCAGCTAGTGAATTCATGTTTGAAGAAACCAGGAACTATGTTAAACAAAGAAAAGCTTTT
GGCAAAACAGTTGCTCACCTACAGACAGTGCAACATAAATTAGCAGAATTAAAAACACAT
ATATGTGTAACCCGAGCATTTGTGGACAACTGTCTCCAGCTGCATGAAGCGAAACGTTTG
GACTCCGCCACTGCTTGCATGGCGAAATATTGGGCATCTGAGTTACAAAATAGTGTAGCT
TACGACTGTGTACAGCTCCATGGAGGTTGGGGATACATGTGGGAGTACCCAATTGCAAAA
GCTTATGTGGATGCCAGAGTTCAGCCAATCTATGGTGGTACAAATGAAATAATGAAGGAG
CTGATTGCAAGAGAGATTGTCTTTGACAAGTAG
Protein Properties
Number of Residues 430
Molecular Weight 47655.275
Theoretical pI 7.803
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Long-chain specific acyl-CoA dehydrogenase, mitochondrial
MAARLLRGSLRVLGGHRAPRQLPAARCSHSGGEERLETPSAKKLTDIGIRRIFSPEHDIF
RKSVRKFFQEEVIPHHSEWEKAGEVSREVWEKAGKQGLLGVNIAEHLGGIGGDLYSAAIV
WEEQAYSNCSGPGFSIHSGIVMSYITNHGSEEQIKHFIPQMTAGKCIGAIAMTEPGAGSD
LQGIKTNAKKDGSDWILNGSKVFISNGSLSDVVIVVAVTNHEAPSPAHGISLFLVENGMK
GFIKGRKLHKMGLKAQDTAELFFEDIRLPASALLGEENKGFYYIMKELPQERLLIADVAI
SASEFMFEETRNYVKQRKAFGKTVAHLQTVQHKLAELKTHICVTRAFVDNCLQLHEAKRL
DSATACMAKYWASELQNSVAYDCVQLHGGWGYMWEYPIAKAYVDARVQPIYGGTNEIMKE
LIAREIVFDK
GenBank ID Protein 177962
UniProtKB/Swiss-Prot ID P28330
UniProtKB/Swiss-Prot Entry Name ACADL_HUMAN
PDB IDs Not Available
GenBank Gene ID M74096
GeneCard ID ACADL
GenAtlas ID ACADL
HGNC ID HGNC:88
References
General References
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