| Nomenclature | Genomic Location |
| Symbol | Acadl | Chromosome | X |
| Name | Acyl-CoA dehydrogenase, long chain | Linkage map | unknown |
| Species | Dracomimus familiaris | Genome Coordinates | ChrX: 47 Mbp |
| Molecular Function |
|
Acyl-CoA + acceptor = 2,3-dehydroacyl-CoA + reduced acceptor
Molecular Function Terms:
binding
carboxylic acid binding
monocarboxylic acid binding
fatty acid binding
cofactor binding
coenzyme binding
fatty-acyl-CoA binding
catalytic activity
oxidoreductase activity
oxidoreductase activity, acting on the CH-CH group of donors
acyl-CoA dehydrogenase activity
|
| Human Disease Association |
|
Defects in ACADL are the cause of acyl-CoA dehydrogenase long-chain deficiency (ACADLD) [MIM:201460].
ACADLD is an inborn error of mitochondrial fatty acid beta-oxidation resulting in hepatic and cardiac
dysfunction, non-ketotic hypoglycemia, hypotonia, muscle weakness.
|
1 GGTACTGTTTCACTATCTGGATGGAGTCCACAAGGAGGAGCTAGAAAGAGAAGGATAGTC
61 CATTTTACCGGGGGGGGGGGGGGAGTATTTTTCTCCCCATTTTTGCTCTTTTCCAGAATG
121 TACGCTAACTGTACTGGACCAGGATTCAGTTTACACTCTGACATTGTCATGCCTTACATT
181 GCCAACTATGGCTCCAAAGAGCAGATTCAGAAGTTCATTCCACAAATGGTAGCAGGAAAA
241 TGTATTGGTGCGATAGCTATGACGGAGCCAGGGGCTGGGAGTGACCTGCAGGGTGTCAGA
301 ACGAATGCGAAAAAGGATGGCAGTGACTGGATTCTAAATGGCAGTAAGGTATTCATTACT
361 AATGGCTGGATGAGTGACTTAGTGATTGTGGTTGCTGTTACAAACCGTGAGGCCCGTTCA
421 CCAGCTCATGGCATAAGCCTCTTCCTAGTGGAAAATGGAATGAAAGGGTTCATTAAAGGG
481 CGCAAGCTGGAGAAAATAGGACTCAAAGCACAGGACACGGCAGAGCTGTTTTTTGAAGAT
541 GTTCGCTTGCCTGCCAGTGCCTTGCTGGGACAGGAGAACAAAGGCTTCTATTATCTTATG
601 GCAGAACTTCCTCAGGAAAGGCTACTGATTGCTGATATGGCAATTGCCAGTTGTGAATTC
661 ATGTTTGAAGAAACAAGAAACTATGTGAAACAGAGAAAAGCCTTTGGAAAAACAATTGCA
721 CATTTGCAGACTGTGCAACACAAGCTAGCGGAATTGAAAACAGAGATCTGCGTAGGTCGG
781 GCATTTCTGGACCACTGCCTCCGACTTCACTCTGGGAAACGGTTGGACTCTGCCACGGCA
841 TCAATGGCGAAATACTGGGCTTCTGATCTCCAAAACAATGTGGCTACTCAGTGTGTACAG
901 CTCCATGGAGGATGGGGATATATGTGGGAGTATCCAATTGCAAAGTGTTTTGTCGATTCT
961 CGTGTTCAACCAATTTATGGTGGTACCAATGAAATAATGAAAGAACTCATTGCCAGAGAT
1021 ATTGTGAGTGACAAATAG
| Predicted Protein Product |
GTVSLSGWSPQGGARKRRIVHFTGGGGGVFFSPFLLFSRMYANCTGPGFSLHSDIVMPYI
ANYGSKEQIQKFIPQMVAGKCIGAIAMTEPGAGSDLQGVRTNAKKDGSDWILNGSKVFIT
NGWMSDLVIVVAVTNREARSPAHGISLFLVENGMKGFIKGRKLEKIGLKAQDTAELFFED
VRLPASALLGQENKGFYYLMAELPQERLLIADMAIASCEFMFEETRNYVKQRKAFGKTIA
HLQTVQHKLAELKTEICVGRAFLDHCLRLHSGKRLDSATASMAKYWASDLQNNVATQCVQ
LHGGWGYMWEYPIAKCFVDSRVQPIYGGTNEIMKELIARDIVSDK
| Protein Alignment to Mouse |
sp|P51174|ACADL_MOUSE Long-chain specific acyl-CoA dehydrogenase,
mitochondrial OS=Mus musculus GN=Acadl PE=2 SV=2
MGI:87866 Acadl acyl-Coenzyme A dehydrogenase, long-chain (Chr 1)
Length = 430
Score = 1440 (512.0 bits), Expect = 3.7e-148, P = 3.7e-148
Identities = 268/304 (88%), Positives = 291/304 (95%)
Query: 41 YANCTGPGFSLHSDIVMPYIANYGSKEQIQKFIPQMVAGKCIGAIAMTEPGAGSDLQGVR 100
Y+NCTGPGFSLHSDIVMPYIANYG+KEQI+KFIPQM AGKCIGAIAMTEPGAGSDLQGVR
Sbjct: 126 YSNCTGPGFSLHSDIVMPYIANYGTKEQIEKFIPQMTAGKCIGAIAMTEPGAGSDLQGVR 185
Query: 101 TNAKKDGSDWILNGSKVFITNGWMSDLVIVVAVTNREARSPAHGISLFLVENGMKGFIKG 160
TNAK+ GSDWILNGSKVFITNGW+SDLVIVVAVTNREARSPAHGISLFLVENGMKGFIKG
Sbjct: 186 TNAKRSGSDWILNGSKVFITNGWLSDLVIVVAVTNREARSPAHGISLFLVENGMKGFIKG 245
Query: 161 RKLEKIGLKAQDTAELFFEDVRLPASALLGQENKGFYYLMAELPQERLLIADMAIASCEF 220
RKL K+G+KAQDTAELFFEDVRLPA+ALLG+ENKGFYYLM ELPQERLLIA++AI++CEF
Sbjct: 246 RKLHKMGMKAQDTAELFFEDVRLPANALLGEENKGFYYLMQELPQERLLIAELAISACEF 305
Query: 221 MFEETRNYVKQRKAFGKTIAHLQTVQHKLAELKTEICVGRAFLDHCLRLHSGKRLDSATA 280
MFEETRNYVKQRKAFGKT+AH+QTVQHKLAELKT ICV RAF+D CL+LH KRLDS +A
Sbjct: 306 MFEETRNYVKQRKAFGKTVAHIQTVQHKLAELKTHICVTRAFVDSCLQLHETKRLDSGSA 365
Query: 281 SMAKYWASDLQNNVATQCVQLHGGWGYMWEYPIAKCFVDSRVQPIYGGTNEIMKELIARD 340
SMAKYWAS+LQN+VA +CVQLHGGWGYMWEYPIAK +VD+RVQPIYGGTNEIMKELIAR
Sbjct: 366 SMAKYWASELQNSVAYECVQLHGGWGYMWEYPIAKAYVDARVQPIYGGTNEIMKELIARQ 425
Query: 341 IVSD 344
IVSD
Sbjct: 426 IVSD 429
|
