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Protein Page:
GDE (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
GDE Multifunctional enzyme acting as 1,4-alpha-D-glucan:1,4- alpha-D-glucan 4-alpha-D-glycosyltransferase and amylo-1,6- glucosidase in glycogen degradation. Defects in AGL are the cause of glycogen storage disease type 3 (GSD3); also known as Forbes disease. GSD3 is a metabolic disorder associated with an accumulation of abnormal glycogen with short outer chains. Three GSD3 types are recognized: GSD type 3A patients lack glycogen debrancher enzyme activity in both liver and muscle, while GSD type 3B patients are enzyme- deficient in liver only. In rare cases, selective loss of only 1 of the 2 debranching activities, glucosidase or transferase, results in GSD type 3C or type 3D, respectively. GSD3 is clinically characterized by hepatomegaly, hypoglycemia, short stature, and variable myopathy. Belongs to the glycogen debranching enzyme family. 3 isoforms of the human protein are produced by alternative splicing. Note: This description may include information from UniProtKB.
Protein type: EC 3.2.1.33; Ubiquitin conjugating system; Hydrolase; Carbohydrate Metabolism - starch and sucrose; EC 2.4.1.25; Transferase
Cellular Component: sarcoplasmic reticulum; cytoplasm; isoamylase complex; nucleolus; inclusion body; nucleus; cytosol
Molecular Function: protein binding; glycogen debranching enzyme activity; 4-alpha-glucanotransferase activity; polyubiquitin binding; amylo-alpha-1,6-glucosidase activity; polysaccharide binding
Biological Process: glycogen biosynthetic process; glycogen catabolic process; response to glucocorticoid stimulus; carbohydrate metabolic process; glucose metabolic process; response to nutrient
Reference #:  P35573 (UniProtKB)
Alt. Names/Synonyms: 4-alpha-glucanotransferase; AGL; amylo-1, 6-glucosidase, 4-alpha-glucanotransferase; Amylo-1,6-glucosidase; amylo-1,6-glucosidase, 4-alpha-glucanotransferase; Amylo-alpha-1,6-glucosidase; Dextrin 6-alpha-D-glucosidase; GDE; Glycogen debrancher; Glycogen debranching enzyme; Oligo-1,4-1,4-glucantransferase
Gene Symbols: AGL
Molecular weight: 174,764 Da
Basal Isoelectric point: 6.31  Predict pI for various phosphorylation states
Select Structure to View Below

GDE

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 2 K5 ___MGHSKQIRILLL
0 2 K17 LLLNEMEKLEKTLFR
0 1 K17 LLLNEMEKLEKTLFR
0 14 Y29-p LFRLEQGyELQFRLG
0 3 Y47 QGKAVTVYTNYPFPG
0 1 K61 GETFNREKFRsLDWE
0 3 S64-p FNREKFRsLDWENPT
0 1 Y106-p NEKSGGGyIVVDPIL
0 1 K151 ESRLRVAKESGYNMI
0 1 S171-p QTLGLSRsCYSLANQ
0 1 R190 PDFSRPNRKYTWNDV
0 1 K203 DVGQLVEKLKKEWNV
0 1 K265 SCDVAEGKYKEKGIP
0 1 K269 AEGKYKEKGIPALIE
0 1 S283-p ENDHHMNsIRKIIWE
0 1 K286 HHMNsIRKIIWEDIF
0 1 K326-ub QENRRVTkSDPNQHL
0 1 T334 SDPNQHLTIIQDPEY
0 1 T420-p GPKLGPVtRKHPLVT
0 1 Y479-p AEPGSEVyLRRELIC
0 2 K492-ub ICWGDSVkLRYGNKP
0 19 Y504-p NKPEDCPyLWAHMkK
0 1 K510-ac PyLWAHMkKYTEITA
0 1 K511 yLWAHMkKYTEITAT
0 5 Y538 TPLHVAEYMLDAARN
0 7 S582-p SLIREAMsAyNsHEE
0 235 Y584-p IREAMsAyNsHEEGR
0 12 S586-p EAMsAyNsHEEGRLV
0 1 Y638-p PIVHRSAyDALPSTT
0 1 K679 SEERFYTKWNPEALP
0 2 K679-ub SEERFYTkWNPEALP
0 1 K761 PKTSFYSKEVPQMCI
0 1 K791 RNTKPYRKDENSING
0 2 K816-ac HIQLNESkIVKQAGV
0 1 K826-ac KQAGVATkGPNEYIQ
0 2 K875-ub TQFSPHFkSGsLAVD
0 1 S878-p SPHFkSGsLAVDNAD
0 1 K889 DNADPILKIPFAsLA
0 1 K889 DNADPILKIPFAsLA
0 2 S894-p ILKIPFAsLAsRLTL
0 1 S897-p IPFAsLAsRLTLAEL
0 1 R898 PFAsLAsRLTLAELN
0 1 T1130-p IILAFAGtLRHGLIP
0 1 K1176 PNGLDILKCPVSRMY
0 1 T1245-p TAGVDEEtGFVYGGN
0 1 K1262 NCGTWMDKMGESDRA
0 1 T1277-p RNRGIPAtPRDGSAV
0 1 S1290-p AVEIVGLsKSAVRWL
0 1 K1302 RWLLELSKKNIFPYH
0 1 K1303 WLLELSKKNIFPYHE
0 1 K1337 KIQDNFEKLFHVSED
0 1 K1351 DPSDLNEKHPNLVHK
0 1 K1399 FTTEKAWKALEIAEk
0 2 K1406-ac KALEIAEkKLLGPLG
0 1 K1406 KALEIAEKKLLGPLG
0 1 K1407 ALEIAEkKLLGPLGM
0 1 K1462 IGYFLRAKLyFsRLM
0 3 Y1464-p YFLRAKLyFsRLMGP
0 2 S1466-p LRAKLyFsRLMGPET
0 1 R1467 RAKLyFsRLMGPETT
0 1 R1467 RAKLyFsRLMGPETT
0 1 K1482 AKTIVLVKNVLSRHY
0 1 K1482 AKTIVLVKNVLSRHY
  mouse

 
K5-ac ___MEHSkQIRILLL
K17-ac LLLNEMEkLEKTLFR
K17-ub LLLNEMEkLEKTLFR
F29 LFRLEQGFELQFRLG
Y47 QGKAVTVYTNYPLPG
K61-ub GEAFNREkFRsLDWE
S64-p FNREkFRsLDWENPT
Y106 NEKSGGGYIVVDPIL
K151-ub ESRLRVAkESGYNMI
S171 QTLGLSRSCYSLADQ
K190-ac PDFSRPSkRYTWSDV
K203-ac DVGQLVEkLKREWNI
K265-ac SCDVADGkYREkGVP
K269-ub ADGkYREkGVPALIE
C283 ENDQHMNCIRkIIWE
K286-ub QHMNCIRkIIWEDIF
K326 QENRRVTKSEPKEHL
K334-ac SEPKEHLkIIQDPEY
T420 GPKLGPVTRKYPLVT
Y479 AEPGSDVYLRRELIC
K492-ub ICWGDSVkLRYGNKP
Y504 NKPEDCPYLWAHMKk
K510 PYLWAHMKkYTEITA
K511-ub YLWAHMKkYTEITAT
Y538 TPLHVAEYMLDAARK
S582 SLIREAMSAYNSHEE
Y584 IREAMSAYNSHEEGR
S586 EAMSAYNSHEEGRLV
Y638 PIVHRSAYDALPSTT
K679-ac AEERFYTkWNPGASP
K679-ub AEERFYTkWNPGASP
K761-ub PKTSFYSkEVPQMCI
K791-ub RNTKPYKkDENSING
K816-ac HIQLHESkIVRQAGV
K826 RQAGVATKGPNEYIQ
K875-ub TQFSSHFkSGSLAVD
S878 SSHFkSGSLAVDNAD
K889-ac DNADPILkIPFASIA
K889-ub DNADPILkIPFASIA
S894 ILkIPFASIASkLTL
S897 IPFASIASkLTLAEL
K898-ac PFASIASkLTLAELN
T1130 IILAFASTLRHGLIP
K1176-ub PNGLDILkCPVSRMY
T1245 TAGIDEETGFVYGGN
K1262-ub NCGTWMDkMGESDRA
T1277 RNRGIPATPRDGSAV
C1290 AVEIVGLCKSAVRWL
K1302-sc RWLLELSkkNIFPYH
K1303-ac WLLELSkkNIFPYHE
K1337-ac KIQNNFEkLFHVSED
K1351-ac DPSDPNEkHPNLVHK
K1399-ac FTAEKAWkALEIAEk
K1406-ac kALEIAEkkLLGPLG
K1406-sc kALEIAEkkLLGPLG
K1407-ub ALEIAEkkLLGPLGM
K1462-sc IGYFLRAkLYFSkMM
Y1464 YFLRAkLYFSkMMGP
S1466 LRAkLYFSkMMGPET
K1467-ac RAkLYFSkMMGPETA
K1467-ub RAkLYFSkMMGPETA
K1482-ub AKTVFLVkNVLSRHY
K1482-sc AKTVFLVkNVLSRHY
  rat

 
K5 ___MEHSKQIRILLL
K17 LLLNEMEKLEKTLFR
K17 LLLNEMEKLEKTLFR
F29 LFRLEQGFELQFRLG
Y47-p QGKAVTVyTNYPFPG
K61 GEAFNREKFRSLDWE
S64 FNREKFRSLDWENPT
Y106 QEKSGGGYIVVDPIL
K151 ESRLRVAKESGYNMI
S171 QALGLSRSCYSLADQ
R190 PDFSRPNRRYTWDDV
K203 DVGQLVEKLKREWNI
K265 SCDVADGKYREKGVP
K269 ADGKYREKGVPALIE
C283 ENDHHMNCIRKIIWE
K286 HHMNCIRKIIWEDIF
K326 QENRRVTKSEPKEHL
K334 SEPKEHLKIIQDPEF
T420 GPKLGPVTRKYPLVT
Y479 AEPGSNVYLRRELIC
K492 ICWGDSVKLRYGNKP
Y504 NKPEDCPYLWAHMKK
K510 PYLWAHMKKYTEITA
K511 YLWAHMKKYTEITAT
Y538-p TPLHVAEyMLDAARK
S582 SLIREAMSAyNSHEE
Y584-p IREAMSAyNSHEEGR
S586 EAMSAyNSHEEGRLV
Y638 PIVHRSAYDALPSTT
K679 AEERFYTKWNPGASP
K679 AEERFYTKWNPGASP
K761 PKTSFYSKEVPQMCI
K791 RNTKPYRKDENSING
K816 RIQLHESKIVKQAGV
K826 KQAGVATKGPNEYIQ
K875 TQFSSHFKSGSLAVD
S878 SSHFKSGSLAVDNSD
K889 DNSDPILKIPFASIA
K889 DNSDPILKIPFASIA
S894 ILKIPFASIASKLTL
S897 IPFASIASKLTLAEL
K898 PFASIASKLTLAELN
T1130 IILAFACTLRHGLIP
K1176 PNGLDILKCPVSRMY
T1245 TVGVDEETGFVYGGN
K1262 NCGTWMDKMGESDRA
T1277 RNRGIPATPRDGSAV
C1290 AVEIVGLCKSAVRWL
K1302 RWLLELSKKNIFPYH
K1303 WLLELSKKNIFPYHE
K1337 KIQNSFEKLFHVSED
K1351 DPSDPNEKHPNLVHK
K1399 FTAEKAWKALEIAEG
G1406 KALEIAEGKLLGPLG
G1406 KALEIAEGKLLGPLG
K1407 ALEIAEGKLLGPLGM
K1462 IGYFLRAKLYFSKMM
Y1464 YFLRAKLYFSKMMGP
S1466 LRAKLYFSKMMGPET
K1467 RAKLYFSKMMGPETA
K1467 RAKLYFSKMMGPETA
K1482 AKTVFLVKNVLSRHY
K1482 AKTVFLVKNVLSRHY
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