3.A.1.203.15 ABCD1 transporter of 766 aas and 6 TMSs. Similar to human patients with X-linked adrenoleukodystrophy (ALD), zebrafish abcd1 mutants have elevated very long chain fatty acid levels, and CNS development was disrupted, with hypomyelination in the spinal cord, abnormal patterning, decreased numbers of oligodendrocytes, and impaired motor function followed by increased cell death (Strachan et al. 2017). Expression of human ABCD1 in zebrafish oligodendrocytes rescued apoptosis in the abcd1 mutant (Strachan et al. 2017).
|
Accession Number: | F1RBC8 |
Protein Name: | ATP-binding cassette, sub-family D (ALD), member 1 |
Length: | 766 |
Molecular Weight: | 86594.00 |
Species: | Danio rerio (Zebrafish) [7955] |
Number of TMSs: | 4 |
Substrate |
long-chain fatty acid |
---|
1: MSVYSKLPSQ LKKPLVKKAV VLLIALYGVK KLSPYFFGKL KGRTSKQVKG ADPLTSNGEP
61: LVEAARKRKR SPAVNREFFD RLIRLLKILF PRLFCKELGL LGFHSLALIS RTFLSIYVAN
121: LDGQIVKTIV KKDPRAFVVE LTKWLLIAIP ATFVNSAIRY LEGQLTLAFR TRLVTHAYML
181: YFSDQTYYRV SNMDGRLANP DQSLTEDVVM FAASVAHLYS NLTKPILDVV VTCYTLIKTA
241: ESKGANTTWP SVIAGIVVAL TAKVLRAFSP RFGKLVAEEA RRKGDLRYMH SRIIANSEEI
301: AFYGGHKIEM LQLQRSYNSL SRQINLILFK RLWYVMLEQF LMKYLWSASG LVMVAVPIIT
361: ATGYSKYDSE DVKQAALDMK EEDLVSERTQ AFTTARSLLN AAADAVERIM VSYKEVTELA
421: GYTARVYEMF EVFEDVRDGV YRRSATEVKP EETGAPQNVT HGMRVEGPLQ IRGQVIDVEQ
481: GIKCENLPII TPTGDVVVSS LNMQVDEGMH LLITGPNGCG KSSLFRILSG LWPVYSGVLY
541: KPSPDHMFYI PQRPYMSVGT LRDQVIYPHS VQEMQEKGIT DRQLEEILQT VSLRYILERE
601: GGWDAVSDWK DVLSGGEKQR MGMARMFYHK PQYALLDECT SAVSIDVEGK IFEAAKDAGI
661: SLLSITHRPS LWKYHSHLLQ FDGEGGWRFE KLDASTRISL QDEKIRLETQ LSGIPKMQQR
721: LTELCRILGE DSSLPTPGDE EEDEEEDEKQ TERDVQSAGK EKDLME