| Nomenclature | Genomic Location |
| Symbol | Acadvl | Chromosome | X |
| Name | Acyl-CoA dehydrogenase,
very long chain | Linkage map | unknown |
| Species | Dracomimus familiaris | Genome Coordinates | ChrX: 10 Mbp |
| Molecular Function |
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Active toward esters of long-chain and very long chain fatty acids such as palmitoyl-CoA, mysritoyl-CoA and stearoyl-CoA. Can accomodate substrate acyl chain lengths as long as 24 carbons, but shows little activity for substrates of less than 12 carbons
Molecular Function Terms:
binding
carboxylic acid binding
monocarboxylic acid binding
fatty acid binding
cofactor binding
coenzyme binding
fatty-acyl-CoA binding
flavin adenine dinucleotide binding
catalytic activity
isomerase activity
oxidoreductase activity
oxidoreductase activity, acting on the CH-CH group of donors
acyl-CoA dehydrogenase activity
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| Human Disease Association |
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Defects in ACADVL are the cause of acyl-CoA dehydrogenase very long chain deficiency (ACADVLD)
[MIM:201475]. ACADVLD is an autosomal recessive disease which leads to impaired long-chain fatty acid
beta-oxidation. It is clinically heterogeneous, with three major phenotypes: a severe childhood form,
with early onset, high mortality, and high incidence of cardiomyopathy; a milder childhood form, with
later onset, usually with hypoketotic hypoglycemia as the main presenting feature, low mortality, and
rare cardiomyopathy; and an adult form, with isolated skeletal muscle involvement, rhabdomyolysis,
and myoglobinuria, usually triggered by exercise or fasting.
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..........................................................AT
3 GCTGCTTCGTAAAGTAAGCCAGTCCTCCGCACTGTGCGGCTCCATTCTGCGGATCCCTGC
63 CGGGCTGTCCAGAGGTCAGCGTCATGCCGGGGCCGTCGTTGCTGTACAGAACCCCCGTCT
123 CTACGCGAGCCAAGCCGCGGAGGCGGTGCTGGAGAAGACGGCCGCAGTCGGCAGTAACAC
183 TGCTGCTAAAGTGGAAAAGACAATTTCTACCGAATCCAAATCGTATGCTGTCAACATTTT
243 CAAGGGGCAGATCGAGACTGCCCAGGTTTTCCCTTATCCCTCAGCCCTGAATGAAGAGCA
303 GGAGCAGTTCCTCCGGGAGCTTGTTGGGCCCGTCGACAAATTCTTTTTGGAAGTGAACGA
363 TGCCGCCAAGAACGACGCCTTGGAGAAGGTCGAGGACGACACCATGCAGGGCCTGAAGGA
423 GATGGGCGCCTTTGGCCTGCAGGTGCCCTCTGACCTGGGCGGCCTCGGCCTCTCAAACAC
483 GCAGTACGCACGGCTGGTCGAGATCGTCGGCACCTACGACCTCGGCGTCGGCATCACCCT
543 GGGAGCCCACCAGTCCATCGGCTTCAAGGGCATTCTGCTCTTTGGGAATCCGGCCCAGAA
603 GGAGAAGTACCTCCCCAAGCTCGCCACTGCCGAGACTATCGCAGCCTTCTGCCTCACCGA
663 ACCGGCCAGCGGCTCAGACGCCGCCTCCATCAAGACCACAGCTGTTCAGTCGTCGTGCGG
723 CAAGTTCTTCACTCTCAACGGGAGCAAGATCTGGATCAGCAACGGCGGTCTGGCTGAGAT
783 CTTCACGGTGTTCGCCAAGACACCCATGAAGGACCCCAAGACGGGGGAGATGAAGGACAA
843 GATCTCTGCCTTCGTGGTGGAGAGGAGCTTCGGAGGAGTGACGAGCGGCCCCCCAGAGAA
903 GAAAATGGGCATCAAGGCGTCGAACACCGCCGAGGTCTACTTTGACAACGTCCGCGTGCC
963 TGCTGACTGCCTGCTGGGCGAATTGGGCGAAGGCTTCAAGGTGGCCATGAACATCCTGAA
1023 TAACGGGCGCTTCGGCATGGCCGCCGCCCTCTCCGGCACCATGAAGGGAGTCATCAACAA
1083 AGCTGTGGACCACGCAGCCAACAGAACCCAGTTCGGAAACAAGATCCACAACTACGGCGT
1143 CATCCAGGAGAAGGTGGCTCGCATGGCCATGCTGCAGTACGTCACCGAGTCGATGGCGTA
1203 CATGATCAGCGGCAACATGGACAGCGGAGCCAAGGAATTCCAGATCGAGGCGGCCATCAG
1263 CAAGATCTTTGCCTCTGAGGCTGCGTGGACTGTGACTGACGAGTGCGTTCAGGTTATGGG
1323 CGGCATGGGTTACATGAAGGACACCGGCCTGGAGAGAGTGATGAGGGATCTGAGGATTTT
1383 CCGAATCTTCGAGGGCACCAACGACATCCTGAGGCTCTTCGTGGCCCTCAACGGCTTCCA
1443 GAACGCTGGTAACCAGTTGAAGAGCTTGCAGAAGGCCCTGAAGAACCCCATCGGCAACGC
1503 CGGGATGCTTGCTGGAGAAATCGCCAAAAGAGCCAAACGAAAGGCCGGTCTGAGCACGGG
1563 TCTGACTCTGCAGGGAACCGTCCACCCCGAGCTGGCTCAGAGCGGAGAACTGACCGCTAA
1623 GGCCATCGAACAGTTTGGAGGTGTCACAGAGGAGCTGCTGCTCAAACACGGGAAGAAGAT
1683 CATTGATGAACAATTTGTCCTGAGCAGAGTGGCAGACTGTGCCATCGACCTCTACGCCAT
1743 GGTGGTGGTCCTGTCCAGTCCTCCGCGTCGCAGGGCTTCACGCGCTCTGAGTGAGGGCAG
1803 CGCCTCGGCTCAGCATGAGAAGATGCTGTGTGAGACCTGGTGTGTGGAGGCCCACGAGAG
1863 GATCCTGAGAGACATCAAGTCGCTGCGCTCCAGTCAGTCCCGACAGCTCTACAAGAACAT
1923 GCGGGCCATCTCTGCCGCCGTGGTGGAGAACGGTGGAGTGGTGGCTCCTCCCGCTCTGGG
1983 TTTCTAG
| Predicted Protein Product |
MLLRKVSQSSALCGSILRIPAGLSRGQRHAGAVVAVQNPRLYASQAAEAVLEKTAAVGSN
TAAKVEKTISTESKSYAVNIFKGQIETAQVFPYPSALNEEQEQFLRELVGPVDKFFLEVN
DAAKNDALEKVEDDTMQGLKEMGAFGLQVPSDLGGLGLSNTQYARLVEIVGTYDLGVGIT
LGAHQSIGFKGILLFGNPAQKEKYLPKLATAETIAAFCLTEPASGSDAASIKTTAVQSSC
GKFFTLNGSKIWISNGGLAEIFTVFAKTPMKDPKTGEMKDKISAFVVERSFGGVTSGPPE
KKMGIKASNTAEVYFDNVRVPADCLLGELGEGFKVAMNILNNGRFGMAAALSGTMKGVIN
KAVDHAANRTQFGNKIHNYGVIQEKVARMAMLQYVTESMAYMISGNMDSGAKEFQIEAAI
SKIFASEAAWTVTDECVQVMGGMGYMKDTGLERVMRDLRIFRIFEGTNDILRLFVALNGF
QNAGNQLKSLQKALKNPIGNAGMLAGEIAKRAKRKAGLSTGLTLQGTVHPELAQSGELTA
KAIEQFGGVTEELLLKHGKKIIDEQFVLSRVADCAIDLYAMVVVLSSPPRRRASRALSEG
SASAQHEKMLCETWCVEAHERILRDIKSLRSSQSRQLYKNMRAISAAVVENGGVVAPPAL
GF
| Protein Alignment to Mouse |
sp|P50544|ACADV_MOUSE Very long-chain specific acyl-CoA dehydrogenase,
mitochondrial OS=Mus musculus GN=Acadvl PE=1 SV=3
MGI:895149 Acadvl acyl-Coenzyme A dehydrogenase, very long chain (Chr 11)
Length = 656
Score = 2185 (774.2 bits), Expect = 4.2e-227, P = 4.2e-227
Identities = 426/648 (65%), Positives = 522/648 (80%)
Query: 16 ILRIPAGLSRGQRHA-GAVVAVQNPRLYASQAAEAVLEKTAAVGSNTAAKVEKTISTESK 74
+LR+ A SR G + RLYA +A +AVL+K + S+ + + EK ESK
Sbjct: 14 LLRLGARSSRSTTVLQGQPRPISAQRLYAREATQAVLDKPETLSSDASTR-EKPARAESK 72
Query: 75 SYAVNIFKGQIETAQVFPYPSALNEEQEQFLRELVGPVDKFFLEVNDAAKNDALEKVEDD 134
S+AV +FKGQ+ QVFPYPS L+EEQ QFL+ELVGPV +FF EVND AKNDALEKVEDD
Sbjct: 73 SFAVGMFKGQLTIDQVFPYPSVLSEEQAQFLKELVGPVARFFEEVNDPAKNDALEKVEDD 132
Query: 135 TMQGLKEMGAFGLQVPSDLGGLGLSNTQYARLVEIVGTYDLGVGITLGAHQSIGFKGILL 194
T+QGLKE+GAFGLQVPS+LGGLGLSNTQYARL EIVG +DLGV +TLGAHQSIGFKGILL
Sbjct: 133 TLQGLKELGAFGLQVPSELGGLGLSNTQYARLAEIVGMHDLGVSVTLGAHQSIGFKGILL 192
Query: 195 FGNPAQKEKYLPKLATAETIAAFCLTEPASGSDAASIKTTAVQSSCGKFFTLNGSKIWIS 254
+G AQ+EKYLP++A+ + +AAFCLTEP+SGSD ASI+++A+ S CGK++TLNGSKIWIS
Sbjct: 193 YGTKAQREKYLPRVASGQALAAFCLTEPSSGSDVASIRSSAIPSPCGKYYTLNGSKIWIS 252
Query: 255 NGGLAEIFTVFAKTPMKDPKTGEMKDKISAFVVERSFGGVTSGPPEKKMGIKASNTAEVY 314
NGGLA+IFTVFAKTP+KD TG +K+KI+AFVVERSFGGVT G PEKKMGIKASNT+EVY
Sbjct: 253 NGGLADIFTVFAKTPIKDAATGAVKEKITAFVVERSFGGVTHGLPEKKMGIKASNTSEVY 312
Query: 315 FDNVRVPADCLLGELGEGFKVAMNILNNGRFGMAAALSGTMKGVINKAVDHAANRTQFGN 374
FD V+VP++ +LGE+G+GFKVA+NILNNGRFGMAA L+GTMK +I KAVDHA NRTQFG+
Sbjct: 313 FDGVKVPSENVLGEVGDGFKVAVNILNNGRFGMAATLAGTMKSLIAKAVDHATNRTQFGD 372
Query: 375 KIHNYGVIQEKVARMAMLQYVTESMAYMISGNMDSGAKEFQIEAAISKIFASEAAWTVTD 434
KIHN+GVIQEK+ARMA+LQYVTESMAYM+S NMD G K+FQIEAAISKIF SEAAW V D
Sbjct: 373 KIHNFGVIQEKLARMAILQYVTESMAYMLSANMDQGFKDFQIEAAISKIFCSEAAWKVAD 432
Query: 435 ECVQVMGGMGYMKDTGLERVMRDLRIFRIFEGTNDILRLFVALNGFQNAGNQLKSLQKAL 494
EC+Q+MGGMG+MK+ G+ERV+RD+RIFRIFEG NDILRLFVAL G + G +L L AL
Sbjct: 433 ECIQIMGGMGFMKEPGVERVLRDIRIFRIFEGANDILRLFVALQGCMDKGKELTGLGNAL 492
Query: 495 KNPIGNAGMLAGEIAKRAKRKAGLSTGLTLQGTVHPELAQSGELTAKAIEQFGGVTEELL 554
KNP GN G+L GE K+ +R+ G+ +GL+L G VHPEL++SGEL +A++QF V E L
Sbjct: 493 KNPFGNVGLLMGEAGKQLRRRTGIGSGLSLSGIVHPELSRSGELAVQALDQFATVVEAKL 552
Query: 555 LKHGKKIIDEQFVLSRVADCAIDLYAMVVVXXXXXXXXXXXXXXEGSASAQHEKMLCETW 614
+KH K I++EQF+L R+AD AIDLYAMVVV EG +AQHEKMLC++W
Sbjct: 553 VKHKKGIVNEQFLLQRLADGAIDLYAMVVVLSRASRSLS-----EGYPTAQHEKMLCDSW 607
Query: 615 CVEAHERILRDIKSLRSS-QSRQLYKNMRAISAAVVENGGVVAPPALG 661
C+EA RI ++ SL+SS Q ++L++N R+IS A+VENGG+V LG
Sbjct: 608 CIEAATRIRENMASLQSSPQHQELFRNFRSISKAMVENGGLVTGNPLG 655
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