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Protein Page:
ADSL (human)
p Phosphorylation
ac Acetylation
me Methylation
m1 Mono-methylation
m2 Di-methylation
m3 Tri-methylation
ub Ubiquitination
sm Sumoylation
ne Neddylation
gl O-GlcNAc
ga O-GalNAc
pa Palmitoylation
ad Adenylylation
sn S-Nitrosylation
ca Caspase cleavage
sc Succinylation

Overview
ADSL Defects in ADSL are the cause of adenylosuccinase deficiency (ADSL deficiency). ADSL deficiency is an autosomal recessive disorder characterized by the accumulation in the body fluids of succinylaminoimidazole-carboxamide riboside (SAICA-riboside) and succinyladenosine (S-Ado). Most children display marked psychomotor delay, often accompanied by epilepsy or autistic features, or both, although some patients may be less profoundly retarded. Occasionally, growth retardation and muscular wasting are also present. Belongs to the lyase 1 family. Adenylosuccinate lyase subfamily. 2 isoforms of the human protein are produced by alternative splicing. Note: This description may include information from UniProtKB.
Protein type: EC 4.3.2.2; Nucleotide Metabolism - purine; Lyase; Amino Acid Metabolism - alanine, aspartate and glutamate
Cellular Component: mitochondrion; cytosol
Molecular Function: adenylosuccinate lyase activity
Biological Process: response to muscle activity; nucleobase, nucleoside and nucleotide metabolic process; metabolic process; purine nucleotide biosynthetic process; purine base metabolic process; 'de novo' IMP biosynthetic process; AMP biosynthetic process; response to starvation; purine ribonucleoside monophosphate biosynthetic process; response to hypoxia; aerobic respiration; protein tetramerization; response to nutrient
Reference #:  P30566 (UniProtKB)
Alt. Names/Synonyms: Adenylosuccinase; Adenylosuccinate lyase; ADSL; AMPS; ASase; ASL; PUR8
Gene Symbols: ADSL
Molecular weight: 54,889 Da
Basal Isoelectric point: 6.68  Predict pI for various phosphorylation states
Select Structure to View Below

ADSL

Protein Structure Not Found.


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Modification Sites and Domains Show Modification Legend
Click here to view phosphorylation modifications only

Modification Sites in Parent Protein, Orthologs, and Isoforms Show Modification Legend
 

Show Multiple Sequence Alignment


 SS 

SS: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.


 MS 

MS: The number of records in which this modification site was assigned using ONLY proteomic discovery-mode mass spectrometry.


       human

 
0 10 S9-p AAGGDHGsPDsYRsP
0 1 S12-p GDHGsPDsYRsPLAS
0 3 S15-p GsPDsYRsPLASRYA
0 1 K75-ub NLENIDFkMAAEEEK
0 1 K82 kMAAEEEKRLRHDVM
0 1 T111-p GIIHLGAtSCYVGDN
0 1 K147-ac SRLADFAkERASLPT
0 1 K170-ub AQLTTVGkRCCLWIQ
0 1 K199 DLRFRGVKGTTGTQA
0 1 K218 LFEGDDHKVEQLDkM
0 1 K224-ub HKVEQLDkMVTEkAG
0 2 K229-ub LDkMVTEkAGFKRAF
0 1 K276-ub IRLLANLkEMEEPFE
0 1 K284 EMEEPFEKQQIGSSA
0 1 K284-ub EMEEPFEkQQIGSSA
0 4 Y294-p IGSSAMPykRNPMRS
0 140 K295-ac GSSAMPykRNPMRSE
0 1 T382 QELPFMATENIIMAM
0 1 S407-p HEKIRVLsQQAAsVV
0 1 S412-p VLsQQAAsVVkQEGG
0 1 K415-ub QQAAsVVkQEGGDND
0 1 Y432 ERIQVDAYFsPIHsQ
0 4 S434-p IQVDAYFsPIHsQLD
0 2 S438-p AYFsPIHsQLDHLLD
0 5 Y466-p RFLEEEVyPLLKPYE
0 1 K470 EEVyPLLKPYESVMK
0 1 K470 EEVyPLLKPYESVMK
  mouse

 
S9 AASGDPGSAESYRSP
S12 GDPGSAESYRSPLAA
S15 GSAESYRSPLAARYA
Q75 NLNNIDFQMAAEEEk
K82-ac QMAAEEEkRLRHDVM
T111 GIIHLGATSCYVGDN
K147 SRLADFAKDRADLPT
K170 AQLTTVGKRCCLWIQ
K199-ub ELRFRGVkGTTGTQA
K218-ub LFEGDHQkVEQLDKM
K224 QkVEQLDKMVTEKAG
K229 LDKMVTEKAGFKRAF
K276 IRLLANLKEMEEPFE
K284-ac EMEEPFEkQQIGSSA
K284 EMEEPFEKQQIGSSA
Y294 IGSSAMPYkRNPMRS
K295-ac GSSAMPYkRNPMRSE
T382-p QELPFMAtENIIMAM
S407 HEKIRVLSQQAAAVV
A412 VLSQQAAAVVKQEGG
K415 QQAAAVVKQEGGDND
Y432-p ERIRADAyFSPIHsQ
S434 IRADAyFSPIHsQLE
S438-p AyFSPIHsQLEHLLD
R466 RFLEEEVRPLLkPYG
K470-ac EEVRPLLkPYGNEMA
K470-ub EEVRPLLkPYGNEMA
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