The first published cDNA sequence of human liver ADSL listed an open reading frame of 1377 nucleotides, starting at nucleotide 78, and encoding a protein of 459 amino acids (4). Correction from C to A of the third nucleotide of this sequence has, however, revealed a first ATG, 75 nucleotides 5' of the initially reported initiation codon (E.A. Fon, EMBL database, accession number X65867). The open reading frame thus comprises 1452 nucleotides, and encodes a protein which is 25 amino acids longer at the N-terminus, containing 484 amino acids.
CCATGGCGGCTGGAGGCGATCATGGTTCGCCCGACAGCTACCGCTCACCTCTTGCCTCCC
60
GCTATGCCAGCCCGGAGATGTGCTTCGTGTTTAGCGACAGGTATAAATTCCGGACATGGC
120
GGCAGCTGTGGCTGTGGCTGGCGGAGGCCGAGCAGACATTGGGTTTGCCTATCACAGATG
180
AACAAATCCAGGAGATGAAATCAAACCTGGAGAACATCGACTTCAAGATGGCAGCTGAGG
240
AAGAGAAACGTTTACGACATGATGTGATGGCTCACGTGCACACATTTGGCCACTGCTGTC
300
CAAAAGCTGCAGGCATTATTCACCTTGGTGCTACTTCTTGCTATGTTGGAGACAATACTG
360
ACTTGATTATTCTTAGAAATGCACTTGACCTGCTTTTGCCAAAGCTTGCCAGAGTGATCT
420
CTCGGCTTGCCGACTTTGCTAAGGAACGAGCCAGTCTACCCACATTAGGTTTCACACATT
480
TCCAGCCTGCACAGCTGACCACAGTTGGGAAACGTTGCTGTCTTTGGATTCAGGATCTTT
540
GCATGGATCTCCAGAACTTGAAGCGTGTCCGAGATGACCTGCGCTTCCGGGGAGTAAAGG
600
GTACCACTGGCACTCAGGCCAGTTTCCTGCAGCTCTTTGAGGGAGATGACCATAAGGTAG
660
AGCAGCTTGACAAGATGGTGACAGAAAAGGCAGGATTTAAGAGAGCTTTCATCATCACAG
720
GGCAGACATATACACGAAAAGTGGATATTGAAGTACTGTCTGTGCTGGCTAGCTTGGGGG
780
CATCAGTGCACAAGATTTGCACCGACATACGCCTCCTGGCAAACCTCAAGGAGATGGAGG
840
AACCCTTTGAAAAACAGCAGATTGGCTCAAGTGCGATGCCATATAAGCGGAATCCCATGC
900
GTTCAGAACGTTGCTGCAGTCTTGCCCGCCACCTGATGACCCTTGTCATGGACCCGCTAC
960
AGACAGCATCTGTCCAGTGGTTTGAACGCACACTGGATGATAGTGCCAACCGACGGATCT
1020
GTTTGGCCGAGGCATTTCTTACCGCAGATACTATATTGAATACGCTGCAGAACATTTCTG
1080
AAGGATTGGTCGTGTACCCCAAAGTAATTGAACGGCGCATTCGGCAAGAGCTGCCTTTCA
1140
TGGCCACAGAGAACATCATCATGGCCATGGTCAAAGCTGGAGGTAGCCGCCAGGATTGCC
1200
ATGAGAAAATCAGAGTGCTTTCTCAGCAGGCAGCTTCTGTGGTTAAGCAGGAAGGGGGTG
1260
ACAATGACCTCATAGAGCGTATCCAGGTTGATGCCTACTTCAGTCCCATTCACTCCCAGT
1320
TGGATCATTTACTGGATCCTTCTTCTTTCACTGGTCGTGCCTCCCAGCAGGTGCAGAGAT
1380
TCTTAGAAGAGGAGGTGTATCCCCTGTTAAAACCATATGAAAGCGTGATGAAGGTGAAAG
1440
CAGAATTATGTCTGTAGAGTTGGAAGAGAATTAAACGAAAATCATTGTTAATTGCTGAGG
1500
CATGAAAATTGTGTTACTATAACGCCTTATTTTACCTCGAGAATTGTTACCTTAAATTAG
1560
TACAGCACTTTCTTCTTCCCATGGTGCTTTCCTGTTTCTCAGTCTCACATTTCTCAACAA
1620
GGCAAAAACAAAGAGCGTTGAAGTTGACTCTGCTCTTGCATAGTAAATGTAGTTCATACT
1680
TGAAAAAAAAAA 1692
ADSL bears sequence homology to a group of enzymes which generate fumarate from different substrates, namely argininosuccinate lyase, aspartase, and class II fumarases (30). A highly conserved 33-nucleotide sequence (in red), encoding amino acids 288 to 298 (GSSAMPYKRNP), is considered the " fumarate lyase " signature.
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