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Protein Page:
ASS1 (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
ASS1 Defects in ASS1 are the cause of citrullinemia type 1 (CTLN1). Citrullinemia belongs to the urea cycle disorders. It is an autosomal recessive disease characterized primarily by elevated serum and urine citrulline levels. Ammonia intoxication is another manifestation. CTLN1 usually manifests in the first few days of life. Affected infants appear normal at birth, but as ammonia builds up in the body they present symptoms such as lethargy, poor feeding, vomiting, seizures and loss of consciousness. Less commonly, a milder CTLN1 form can develop later in childhood or adulthood. Belongs to the argininosuccinate synthase family. Type 1 subfamily. Note: This description may include information from UniProtKB.
Protein type: Ligase; Mitochondrial; Endoplasmic reticulum; Amino Acid Metabolism - alanine, aspartate and glutamate; Amino Acid Metabolism - arginine and proline; EC 6.3.4.5
Cellular Component: cytoplasm; cytosol
Molecular Function: amino acid binding; protein binding; argininosuccinate synthase activity; ATP binding
Biological Process: positive regulation of nitric oxide biosynthetic process; arginine biosynthetic process; citrulline metabolic process; aspartate metabolic process; argininosuccinate metabolic process; urea cycle
Reference #:  P00966 (UniProtKB)
Alt. Names/Synonyms: Argininosuccinate synthase; argininosuccinate synthase 1; argininosuccinate synthetase 1; ASS; ASS1; ASSY; Citrulline--aspartate ligase; citrulline-aspartate ligase; CTLN1
Gene Symbols: ASS1
Molecular weight: 46,530 Da
Basal Isoelectric point: 8.08  Predict pI for various phosphorylation states
Select Structure to View Below

ASS1

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 1 K25-m2 SCILVWLkEQGYDVI
0 2 K41 YLANIGQKEDFEEAR
0 1 K41 YLANIGQKEDFEEAR
0 1 K41 YLANIGQKEDFEEAR
0 1 K58 ALKLGAKKVFIEDVS
0 1 K58 ALKLGAKKVFIEDVS
0 2 Y83-p AIQSSALyEDRyLLG
0 2 Y87-p SALyEDRyLLGTsLA
0 1 S92-p DRyLLGTsLARPCIA
0 2 K112 IAQREGAKyVSHGAt
0 2 K112 IAQREGAKyVSHGAt
0 1 K112 IAQREGAKyVSHGAt
0 1 Y113-p AQREGAKyVSHGAtG
0 1 T119-p KyVSHGAtGkGNDQV
0 1 K121 VSHGAtGKGNDQVRF
0 4 K121-ub VSHGAtGkGNDQVRF
0 1 K121 VSHGAtGKGNDQVRF
0 2 S131-p DQVRFELsCysLAPQ
0 30 Y133-p VRFELsCysLAPQIK
0 7 S134-p RFELsCysLAPQIKV
0 1 K140 ysLAPQIKVIAPWRM
0 2 Y151 PWRMPEFYNRFKGRN
0 1 K155 PEFYNRFKGRNDLME
0 4 Y163-p GRNDLMEyAkQHGIP
0 4 K165-ac NDLMEyAkQHGIPIP
0 1 K165 NDLMEyAKQHGIPIP
0 1 K165-sc NDLMEyAkQHGIPIP
0 4 T174-p HGIPIPVtPKNPWsM
0 1 K176 IPIPVtPKNPWsMDE
0 3 N177 PIPVtPKNPWsMDEN
0 7 S180-p VtPKNPWsMDENLMH
0 1 K199 AGILENPKNQAPPGL
0 1 K209-ac APPGLYTkTQDPAKA
0 2 K209-ub APPGLYTkTQDPAKA
0 1 K209 APPGLYTKTQDPAKA
0 1 T210 PPGLYTkTQDPAKAP
0 1 K215 TkTQDPAKAPNtPDI
0 1 K215 TkTQDPAKAPNtPDI
0 28 T219-p DPAKAPNtPDILEIE
0 30 K228-ac DILEIEFkKGVPVKV
0 1 K228 DILEIEFKKGVPVKV
0 1 K228-sc DILEIEFkKGVPVKV
0 4 K229 ILEIEFkKGVPVKVT
0 1 K234 FkKGVPVKVTNVKDG
0 2 K239 PVKVTNVKDGTTHQT
0 1 K239 PVKVTNVKDGTTHQT
0 1 K239 PVKVTNVKDGTTHQT
0 1 K260 YLNEVAGKHGVGRID
0 1 K260 YLNEVAGKHGVGRID
0 1 K277 ENRFIGMKSRGIYET
0 1 Y291-p TPAGTILyHAHLDIE
0 1 K310 DREVRKIKQGLGLKF
0 1 K310 DREVRKIKQGLGLKF
0 32 Y322-p LKFAELVyTGFWHSP
0 4 K340 FVRHCIAKSQERVEG
0 3 K340 FVRHCIAKSQERVEG
0 1 K348 SQERVEGKVQVsVLK
0 2 S352-p VEGKVQVsVLKGQVy
0 1 K355 KVQVsVLKGQVyILG
0 1 K355 KVQVsVLKGQVyILG
0 2 Y359-p sVLKGQVyILGRESP
0 11 Y383-p SMNVQGDyEPTDATG
0 2 Y402-p NSLRLKEyHRLQSKV
0 1 K408 EyHRLQSKVTAK___
  mouse

 
K25 SCILVWLKEQGYDVI
K41-ac YLANIGQkEDFEEAR
K41-ub YLANIGQkEDFEEAR
K41-sc YLANIGQkEDFEEAR
K58-ub ALKLGAKkVFIEDVS
K58-sc ALKLGAKkVFIEDVS
Y83 AVQSSALYEDRYLLG
Y87 SALYEDRYLLGTsLA
S92-p DRYLLGTsLARPCIA
K112-ac IAQREGAkYVSHGAT
K112-ub IAQREGAkYVSHGAT
K112-sc IAQREGAkYVSHGAT
Y113 AQREGAkYVSHGATG
T119 kYVSHGATGkGNDQV
K121-ac VSHGATGkGNDQVRF
K121-ub VSHGATGkGNDQVRF
K121-sc VSHGATGkGNDQVRF
T131 DQVRFELTCysLAPQ
Y133-p VRFELTCysLAPQIk
S134-p RFELTCysLAPQIkV
K140-ub ysLAPQIkVIAPWRM
Y151-p PWRMPEFyNRFkGRN
K155-ub PEFyNRFkGRNDLME
Y163 GRNDLMEYAkQHGIP
K165-ac NDLMEYAkQHGIPIP
K165-ub NDLMEYAkQHGIPIP
K165-sc NDLMEYAkQHGIPIP
T174-p HGIPIPVtPksPWsM
K176-ub IPIPVtPksPWsMDE
S177-p PIPVtPksPWsMDEN
S180-p VtPksPWsMDENLMH
K199-ub AGILENPkNQAPPGL
K209 APPGLYTKtQDPAkA
K209-ub APPGLYTktQDPAkA
K209-sc APPGLYTktQDPAkA
T210-p PPGLYTktQDPAkAP
K215-ac TktQDPAkAPNsPDV
K215-ub TktQDPAkAPNsPDV
S219-p DPAkAPNsPDVLEIE
K228-ac DVLEIEFkkGVPVkV
K228-ub DVLEIEFkkGVPVkV
K228-sc DVLEIEFkkGVPVkV
K229-ac VLEIEFkkGVPVkVT
K234-ub FkkGVPVkVTNIkDG
K239-ac PVkVTNIkDGTTRTT
K239-ub PVkVTNIkDGTTRTT
K239-sc PVkVTNIkDGTTRTT
K260-ac YLNEVAGkHGVGRID
K260-ub YLNEVAGkHGVGRID
K277-ub ENRFIGMkSRGIYET
Y291 TPAGTILYHAHLDIE
K310-ub DREVRKIkQGLGLKF
K310-sc DREVRKIkQGLGLKF
Y322-p LKFAELVyTGFWHSP
K340-ac FVRHCIQkSQERVEG
K340-ub FVRHCIQkSQERVEG
K348-sc SQERVEGkVQVSVFk
S352 VEGkVQVSVFkGQVY
K355-ac kVQVSVFkGQVYILG
K355-sc kVQVSVFkGQVYILG
Y359 SVFkGQVYILGRESP
Y383-p SMNVQGDyEPIDATG
Y402 NSLRLKEYHRLQSkV
K408-ub EYHRLQSkVTAK___
  rat

 
K25 SCILVWLKEQGYDVI
K41-ac YLANIGQkEDFEEAR
K41 YLANIGQKEDFEEAR
K41 YLANIGQKEDFEEAR
K58 ALKLGAKKVFIEDVS
K58 ALKLGAKKVFIEDVS
Y83-p AVQSSALyEDRyLLG
Y87-p SALyEDRyLLGTSLA
S92 DRyLLGTSLARPCIA
K112-ac IAQREGAkYVSHGAT
K112 IAQREGAKYVSHGAT
K112 IAQREGAKYVSHGAT
Y113 AQREGAkYVSHGATG
T119 kYVSHGATGKGNDQV
K121 VSHGATGKGNDQVRF
K121 VSHGATGKGNDQVRF
K121 VSHGATGKGNDQVRF
T131 DQVRFELTCYSLAPQ
Y133 VRFELTCYSLAPQIK
S134 RFELTCYSLAPQIKV
K140 YSLAPQIKVIAPWRM
Y151 PWRMPEFYNRFKGRN
K155 PEFYNRFKGRNDLME
Y163-p GRNDLMEyAkQHGIP
K165-ac NDLMEyAkQHGIPIP
K165 NDLMEyAKQHGIPIP
K165 NDLMEyAKQHGIPIP
T174 HGIPIPVTPKSPWSM
K176 IPIPVTPKSPWSMDE
S177 PIPVTPKSPWSMDEN
S180 VTPKSPWSMDENLMH
K199 AGILENPKNQAPPGL
K209-ac APPGLYTkTQDPAkA
K209 APPGLYTKTQDPAkA
K209 APPGLYTKTQDPAkA
T210 PPGLYTkTQDPAkAP
K215-ac TkTQDPAkAPNtPDV
K215 TkTQDPAKAPNtPDV
T219-p DPAkAPNtPDVLEIE
K228-ac DVLEIEFkKGVPVKV
K228 DVLEIEFKKGVPVKV
K228 DVLEIEFKKGVPVKV
K229 VLEIEFkKGVPVKVT
K234 FkKGVPVKVTNVKDG
K239 PVKVTNVKDGTTHST
K239 PVKVTNVKDGTTHST
K239 PVKVTNVKDGTTHST
K260 YLNEVAGKHGVGRID
K260 YLNEVAGKHGVGRID
K277 ENRFIGMKSRGIYET
Y291 TPAGTILYHAHLDIE
K310 DREVRKIKQGLGLKF
K310 DREVRKIKQGLGLKF
Y322 LKFAELVYTGFWHSP
K340 FVRHCIDKSQERVEG
K340 FVRHCIDKSQERVEG
K348 SQERVEGKVQVSVFK
S352 VEGKVQVSVFKGQVY
K355 KVQVSVFKGQVYILG
K355 KVQVSVFKGQVYILG
Y359 SVFKGQVYILGRESP
Y383 SMNVQGDYEPIDATG
Y402 NSLRLKEYHRLQSKV
K408 EYHRLQSKVTAK___
  cow

 
K25 SCILVWLKEQGYDVI
K41 YLANIGQKEDFEEAR
K41 YLANIGQKEDFEEAR
K41 YLANIGQKEDFEEAR
K58 ALKLGAKKVFIEDIS
K58 ALKLGAKKVFIEDIS
Y83 AIQSSALYEDRYLLG
Y87 SALYEDRYLLGTSLA
S92 DRYLLGTSLARPCIA
K112 IAQREGAKYVSHGAT
K112 IAQREGAKYVSHGAT
K112 IAQREGAKYVSHGAT
Y113 AQREGAKYVSHGATG
T119 KYVSHGATGKGNDQI
K121 VSHGATGKGNDQIRF
K121 VSHGATGKGNDQIRF
K121 VSHGATGKGNDQIRF
T131 DQIRFELTCYSLAPQ
Y133 IRFELTCYSLAPQIK
S134 RFELTCYSLAPQIKV
K140 YSLAPQIKVIAPWRM
Y151 PWRMPEFYNRFQGRN
Q155 PEFYNRFQGRNDLME
Y163 GRNDLMEYAKQHGIP
K165 NDLMEYAKQHGIPVP
K165 NDLMEYAKQHGIPVP
K165 NDLMEYAKQHGIPVP
T174 HGIPVPVTPKNPWSM
K176 IPVPVTPKNPWSMDE
N177 PVPVTPKNPWSMDEN
S180 VTPKNPWSMDENLMH
K199 AGILENPKNQAPPGL
K209 APPGLYTKTQDPAKA
K209 APPGLYTKTQDPAKA
K209 APPGLYTKTQDPAKA
T210 PPGLYTKTQDPAKAP
K215 TKTQDPAKAPNSPDM
K215 TKTQDPAKAPNSPDM
S219 DPAKAPNSPDMLEIE
K228 DMLEIEFKKGVPVKV
K228 DMLEIEFKKGVPVKV
K228 DMLEIEFKKGVPVKV
K229 MLEIEFKKGVPVKVT
K234 FKKGVPVKVTNVGDG
G239 PVKVTNVGDGTTHST
G239 PVKVTNVGDGTTHST
G239 PVKVTNVGDGTTHST
K260 YLNEVAGKHGVGRID
K260 YLNEVAGKHGVGRID
K277 ENRFIGMKSRGIYET
Y291 TPAGTILYHAHLDIE
K310 DREVRKIKQGLGLKF
K310 DREVRKIKQGLGLKF
Y322 LKFAELVYTGFWHsP
K340 FVRHCIAKSQERVEG
K340 FVRHCIAKSQERVEG
K348 SQERVEGKVQVSVFK
S352 VEGKVQVSVFKGQVY
K355 KVQVSVFKGQVYILG
K355 KVQVSVFKGQVYILG
Y359 SVFKGQVYILGRESP
Y383 SMNVQGDYEPVDATG
Y402 NSLRLKEYHRLQNKV
K408 EYHRLQNKVTAK___
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