Protein Adsl

URN urn:agi-llid:158
Connectivity 18
Name Adsl
Description adenylosuccinate lyase
Notes Adenylsuccinate lyase is involved in both de novo synthesis of purines and formation of adenosine monophosphate from inosine monophosphate. It catalyzes two reactions in AMP biosynthesis: the removal of a fumarate from succinylaminoimidazole carboxamide (SAICA) ribotide to give aminoimidazole carboxamide ribotide (AICA) and removal of fumarate from adenylosuccinate to give AMP. Adenylosuccinase deficiency results in succinylpurinemic autism, psychomotor retardation, and , in some cases, growth retardation associated with muscle wasting and epilepsy. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq]

Pathway de novo purine biosynthetic pathway
'Copy of 'de novo purine biosynthetic pathway'
de novo purine biosynthetic pathwaylatest
Copy of 'de novo purine biosynthetic pathwaylatest'
Copy of 'de novo purine biosynthetic pathwaylatest'
de novo purine biosynthetic pathway

GO Molecular Function catalytic activity
lyase activity
(S)-2-(5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamido)succinate AMP-lyase (fumarate-forming) activity
N6-(1,2-dicarboxyethyl)AMP AMP-lyase (fumarate-forming) activity

GO Cellular Component soluble fraction
cytoplasm
cytosol
mitochondrion

GO Biological Process purine nucleotide metabolic process
purine nucleotide biosynthetic process
purine ribonucleotide biosynthetic process
purine ribonucleoside monophosphate biosynthetic process
AMP biosynthetic process
'de novo' IMP biosynthetic process
aerobic respiration
response to nutrient
response to hypoxia
protein tetramerization

Ariadne Ontology Purine metabolism

Group Purine metabolism
catalytic activity
purine nucleotide metabolic process
purine nucleotide biosynthetic process
purine ribonucleotide biosynthetic process
purine ribonucleoside monophosphate biosynthetic process
AMP biosynthetic process
'de novo' IMP biosynthetic process
aerobic respiration
response to nutrient
response to hypoxia
protein tetramerization
lyase activity
(S)-2-(5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamido)succinate AMP-lyase (fumarate-forming) activity
N6-(1,2-dicarboxyethyl)AMP AMP-lyase (fumarate-forming) activity
soluble fraction
cytoplasm
cytosol
mitochondrion

MedScan ID 158

Hugo ID 291

Human chromosome position 22q13.1

LocusLink ID 158
11564
315150

Alias ASL
AMPS
ASASE
RP5-1042K10.8
adenylosuccinate lyase
adenylosuccinase
adenylosuccinate lyase 1
null
test
ADSL
OTTHUMP00000028724
succino AMP-lyase
succino AMP-lyases

Organism Homo sapiens
Mus musculus
Rattus norvegicus

GenBank ID NC_000022
NM_000026
NP_000017
NM_001123378
NP_001116850
NT_011520
NW_927628
NW_001838745
NG_007993
AC_000065
AC_000154
AF106656
AAC83935
AL022238
CAI18983
CAQ08929
CAQ08930
CH471095
EAW60375
EAW60376
EAW60377
AF067853
AAC21560
AF067854
AAC21561
AK225042
AK291091
BAF83780
AK298187
BAG60458
AK300705
BAG62384
AK308662
BC000253
AAH00253
BC018881
BP298407
CR456368
CAG30254
CR590245
CR594266
CR594779
CR599886
CR603727
CR609122
CR610943
CR613803
CR614973
CR616687
CR616911
CR619354
CR622395
CR623741
S60710
AAC60603
X65867
CAA46696
CAA46697
P30566
Q71UA4
CU012935
CAK54366
CU013223
CAK54665
DQ893326
ABM84252
DQ896642
ABM87641
NC_000081
NM_009634
NP_033764
NT_039621
NW_001030577
AC_000037
AC125540
AC141880
CH466550
EDL04592
AK049372
BAC33717
AK051111
BAC34529
AK152092
BAE30940
AK166019
BAE38524
AK168906
BAE40720
AK170216
AK171725
BAE42633
AK181586
AK190788
AK201011
BC020187
AAH20187
U20225
AAB60684
U20226
P54822
Q3TMB8
Q8VCD4
NC_005106
NM_001130503
NW_047780
NW_001084859
AC_000075
CH473950
EDM15741

OMIM ID 608222
103050

Mouse chromosome position 15 46.0 cM

GO ID 0070626
0004018
0016829
0006167
0051262
0006164
0009168
0005737
0005829
0003824
0009060
0006163
0009152
0007584
0005739
0005625
0001666
0006189

Rat chromosome position 7q34

Swiss-Prot Accession P30566
Q71UA4
Q8VCD4
P54822
Q3TMB8
B0QY76
O75495
Q5TI34

Unigene ID Hs.75527
Mm.38151
Rn.23276

KEGG ID hsa:158
mmu:11564
rno:315150

EC Number 4.3.2.2

Swiss-Prot ID PUR8_HUMAN
Q8VCD4_MOUSE
B0QY76_HUMAN

Cell Localization Cytoplasm

IPI ID IPI00220887
IPI00942092
IPI00265471
IPI00026904
IPI00878077
IPI00308217
IPI00361216

Homologene ID 12

RGD ID 1307617

MGI ID 103202