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Protein Page:
PFKM (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
PFKM phosphofructokinase, muscle type. An ubiquitous metabolic enzyme involved in the synthesis and degradation of fructose 2,6-bisphosphate. Key control step of glycolysis. An allosteric enzyme activated by ADP, AMP, or fructose bisphosphate and inhibited by ATP or citrate. Activity: ATP D-fructose 6-phosphate = ADP D-fructose 1,6-bisphosphate. The holoenzyme consists of 4 subunits. The liver and muscle enzymes are homo-tetramers of four liver or muscle isoforms, respectively. The red blood cell enzyme consists hetero-tetramers of the muscle and liver isoforms. A subunit composition with a higher proportion of platelet type subunits is found in platelets, brain and fibroblasts. Defects in PFKM are the cause of glycogen storage disease VII (GSD-VII) also known as Tarui disease. Two alternatively spliced isoforms have been described. Note: This description may include information from UniProtKB.
Protein type: Carbohydrate Metabolism - glycolysis and gluconeogenesis; EC 2.7.1.11; Carbohydrate Metabolism - pentose phosphate pathway; Carbohydrate Metabolism - galactose; Carbohydrate Metabolism - fructose and mannose; Kinase, other
Cellular Component: 6-phosphofructokinase complex; apical plasma membrane; cytosol
Molecular Function: protein C-terminus binding; identical protein binding; protein binding; metal ion binding; kinase binding; 6-phosphofructokinase activity; ATP binding
Biological Process: muscle maintenance; glycolysis; carbohydrate metabolic process; carbohydrate phosphorylation; glucose metabolic process; positive regulation of insulin secretion; fructose 6-phosphate metabolic process; glucose homeostasis; protein oligomerization
Reference #:  P08237 (UniProtKB)
Alt. Names/Synonyms: 6-phosphofructo-1-kinase; 6-phosphofructokinase, muscle type; GSD7; K6PF; MGC8699; PFK-1; PFK-A; PFK1; PFKA; PFKM; PFKX; Phosphofructo-1-kinase isozyme A; Phosphofructokinase 1; phosphofructokinase, muscle; phosphofructokinase, polypeptide X; Phosphofructokinase-M; Phosphohexokinase
Gene Symbols: PFKM
Molecular weight: 85,183 Da
Basal Isoelectric point: 8.23  Predict pI for various phosphorylation states
Select Structure to View Below

PFKM

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

► Hide Isoforms
 
0 1 - gap
0 1 - gap
0 2 - gap
0 1 K10-ac HEEHHAAkTLGIGkA
0 1 K16-ub AkTLGIGkAIAVLTS
0 1 T82-p MMLQLGGtVIGSARC
0 144 Y103-p EGRLRAAyNLVKRGI
0 1 K107 RAAyNLVKRGITNLC
0 1 S130-p TGADTFRsEWsDLLs
0 1 S133-p DTFRsEWsDLLsDLQ
0 1 S137-p sEWsDLLsDLQkAGk
0 4 K141-ub DLLsDLQkAGkITDE
0 1 K144-ub sDLQkAGkITDEEAT
0 1 S198-p AITTTAQsHQRTFVL
0 1 K272-ub GAIDKNGkPITSEDI
0 1 K280 PITSEDIKNLVVKRL
0 2 T304 GHVQRGGTPSAFDRI
0 1 T328-p VMALLEGtPDtPACV
0 1 T331-p LLEGtPDtPACVVSL
0 2 K360-ac QVTKDVTkAMDEKkF
0 1 K360-ub QVTKDVTkAMDEKkF
0 2 K366-ac TkAMDEKkFDEALkL
0 1 K366-ub TkAMDEKkFDEALkL
0 1 K372 KkFDEALKLRGRsFM
0 4 K372-ub KkFDEALkLRGRsFM
1 9 S377-p ALkLRGRsFMNNWEV
0 1 S398-p VRPPVSKsGsHtVAV
0 1 S400-p PPVSKsGsHtVAVMN
0 1 T402-p VSKsGsHtVAVMNVG
0 1 K445-ub DGFEGLAkGQIEEAG
0 1 K466-ub WTGQGGSkLGTKRtL
0 1 T472-p SkLGTKRtLPKksFE
0 1 K476-ub TKRtLPKksFEQISA
0 1 S477-p KRtLPKksFEQISAN
0 1 Y502-p IIGGFEAyTGGLELM
0 12 K564-ac KQSAAGTkRRVFIIE
0 3 Y576-p IIETMGGyCGYLATM
0 1 K615-ub NVEHLVQkMKTTVKR
0 1 K656-ub KGIFDSRkNVLGHMQ
0 5 S667-p GHMQQGGsPTPFDRN
0 3 K678-ub FDRNFATkMGAkAMN
0 1 K682-ub FATkMGAkAMNWMSG
0 1 K727-ac FQPVAELkDQTDFEH
0 1 K727-ub FQPVAELkDQTDFEH
0 1 Y755-p ILKILAKyEIDLDTS
2 19 S775-p EHITRKRsGEAAV__
  PFKM iso2  
- gap
- gap
- gap
K10 HEEHHAAKTLGIGKA
K16 AKTLGIGKAIAVLTS
T82 MMLQLGGTVIGSARC
Y103 EGRLRAAYNLVKRGI
K107 RAAYNLVKRGITNLC
S130 TGADTFRSEWSDLLS
S133 DTFRSEWSDLLSDLQ
S137 SEWSDLLSDLQKAGK
K141 DLLSDLQKAGKITDE
K144 SDLQKAGKITDEEAT
S198 AITTTAQSHQRTFVL
K272-ub GAIDKNGkPITSEDI
K280-ub PITSEDIkNGSRMGV
- gap
T297 VMALLEGTPDTPACV
T300 LLEGTPDTPACVVSL
K329 QVTKDVTKAMDEKKF
K329 QVTKDVTKAMDEKKF
K335 TKAMDEKKFDEALKL
K335 TKAMDEKKFDEALKL
K341 KKFDEALKLRGRSFM
K341 KKFDEALKLRGRSFM
S346 ALKLRGRSFMNNWEV
S367 VRPPVSKSGSHTVAV
S369 PPVSKSGSHTVAVMN
T371 VSKSGSHTVAVMNVG
K414 DGFEGLAKGQIEEAG
K435 WTGQGGSKLGTKRTL
T441 SKLGTKRTLPKKSFE
K445 TKRTLPKKSFEQISA
S446 KRTLPKKSFEQISAN
Y471 IIGGFEAYTGGLELM
K533 KQSAAGTKRRVFIIE
Y545 IIETMGGYCGYLATM
K584 NVEHLVQKMKTTVKR
K625 KGIFDSRKNVLGHMQ
S636 GHMQQGGSPTPFDRN
K647 FDRNFATKMGAKAMN
K651 FATKMGAKAMNWMSG
K696 FQPVAELKDQTDFEH
K696 FQPVAELKDQTDFEH
Y724 ILKILAKYEIDLDTS
S744 EHITRKRSGEAAV__
  PFKM iso3  
K49-ub PPKTDILkSLDTMDD
T59-p DTMDDPDtVGsIPVF
S62-p DDPDtVGsIPVFKTE
K81 HEEHHAAKTLGIGKA
K87 AKTLGIGKAIAVLTS
T153 MMLQLGGTVIGSARC
Y174 EGRLRAAYNLVKRGI
K178 RAAYNLVKRGITNLC
S201 TGADTFRSEWSDLLS
S204 DTFRSEWSDLLSDLQ
S208 SEWSDLLSDLQKAGK
K212 DLLSDLQKAGKITDE
K215 SDLQKAGKITDEEAT
S269 AITTTAQSHQRTFVL
K343 GAIDKNGKPITSEDI
K351 PITSEDIKNLVVKRL
T375 GHVQRGGTPSAFDRI
T399 VMALLEGTPDTPACV
T402 LLEGTPDTPACVVSL
K431 QVTKDVTKAMDEKKF
K431 QVTKDVTKAMDEKKF
K437 TKAMDEKKFDEALKL
K437 TKAMDEKKFDEALKL
K443 KKFDEALKLRGRSFM
K443 KKFDEALKLRGRSFM
S448 ALKLRGRSFMNNWEV
S469 VRPPVSKSGSHTVAV
S471 PPVSKSGSHTVAVMN
T473 VSKSGSHTVAVMNVG
K516 DGFEGLAKGQIEEAG
K537 WTGQGGSKLGTKRTL
T543 SKLGTKRTLPKKSFE
K547 TKRTLPKKSFEQISA
S548 KRTLPKKSFEQISAN
Y573 IIGGFEAYTGGLELM
K635 KQSAAGTKRRVFIIE
Y647 IIETMGGYCGYLATM
K686 NVEHLVQKMKTTVKR
K727 KGIFDSRKNVLGHMQ
S738 GHMQQGGSPTPFDRN
K749 FDRNFATKMGAKAMN
K753 FATKMGAKAMNWMSG
K798 FQPVAELKDQTDFEH
K798 FQPVAELKDQTDFEH
Y826 ILKILAKYEIDLDTS
S846 EHITRKRSGEAAV__
  mouse

 
- gap
- gap
- gap
K10 HEEHHAAKTLGIGKA
K16 AKTLGIGKAIAVLTS
T82 MMLQLGGTVIGSARC
H103 EGRLRAAHNLVkRGI
K107-ub RAAHNLVkRGITNLC
S130 TGADTFRSEWSDLLN
S133 DTFRSEWSDLLNDLQ
N137 SEWSDLLNDLQkDGK
K141-ub DLLNDLQkDGKITAE
K144 NDLQkDGKITAEEAT
S198 AITTTAQSHQRTFVL
K272 GAIDKNGKPITSEDI
K280 PITSEDIKNLVVKRL
T304-p GHVQRGGtPSAFDRI
T328 VMALLEGTPDTPACV
T331 LLEGTPDTPACVVSL
K360 QVTKDVTKAMDEKRF
K360 QVTKDVTKAMDEKRF
R366 TKAMDEKRFDEAIkL
R366 TKAMDEKRFDEAIkL
K372 KRFDEAIKLRGRsFM
K372-ub KRFDEAIkLRGRsFM
S377-p AIkLRGRsFMNNWEV
G398 VRPPVSKGGLHTVAV
L400 PPVSKGGLHTVAVMN
T402 VSKGGLHTVAVMNVG
K445 DGFEGLAKGQIEEAG
K466 WTGQGGSKLGTKRTL
T472 SKLGTKRTLPKKNLE
K476 TKRTLPKKNLEQISA
N477 KRTLPKKNLEQISAN
Y502 IIGGFEAYTGGLELM
K564 KQSAAGTKRRVFIIE
Y576 IIETMGGYCGYLATM
K615 NVEHLVQKMKTTVKR
K656 KGIFDSRKNVLGHMQ
S667-p GHMQQGGsPTPFDRN
K678-ub FDRNFATkMGAKAMN
K682 FATkMGAKAMNWMSG
K727 FQPVTELKDQTDFEH
K727 FQPVTELKDQTDFEH
Y755 ILKILAKYEIDLDTS
S775-p EHISRKRsGEAAV__
  rat

 
- gap
- gap
- gap
K10-ac HEEHHEAkTLGIGKA
K16 AkTLGIGKAIAVLTS
T82 MMLQLGGTVIGSARC
H103 EGRLRAAHNLVKRGI
K107 RAAHNLVKRGITNLC
S130 TGADTFRSEWsDLLN
S133-p DTFRSEWsDLLNDLQ
N137 SEWsDLLNDLQKDGK
K141 DLLNDLQKDGKITAE
K144 NDLQKDGKITAEERT
S198 AITTTAQSHQRTFVL
K272 GAIDKNGKPITSEDI
K280 PITSEDIKNLVVKRL
T304-p GHVQRGGtPSAFDRI
T328 VMALLEGTPDTPACV
T331 LLEGTPDTPACVVSL
K360-ac QVTKDVTkAMDEKRF
K360 QVTKDVTKAMDEKRF
R366 TkAMDEKRFDEAIkL
R366 TkAMDEKRFDEAIkL
K372-ac KRFDEAIkLRGRsFM
K372 KRFDEAIKLRGRsFM
S377-p AIkLRGRsFMNNWEV
G398 VRPPVSKGGLHTVAV
L400 PPVSKGGLHTVAVMN
T402 VSKGGLHTVAVMNVG
K445 DGFEGLAKGQIEEAG
K466 WTGQGGSKLGTKRTL
T472 SKLGTKRTLPKKNLE
K476 TKRTLPKKNLEQISA
N477 KRTLPKKNLEQISAN
Y502 IIGGFEAYTGGLELM
K564 KQSAAGTKRRVFIIE
Y576 IIETMGGYCGYLATM
K615 NVEHLVQKMKTTVKR
K656 KGIFDSRKNVLGHMQ
N667 GHMQQGGNPTPFDRN
K678 FDRNFATKMGAKATN
K682 FATKMGAKATNWMSG
K727-ac FQPVTELkDQTDFEH
K727 FQPVTELKDQTDFEH
Y755 ILKILAKYEIDLDTS
S775-p EHISRKRsGEAAV__
  rabbit

 
- gap
- gap
- gap
R10 HEEHHAARTLGVGKA
K16 ARTLGVGKAIAVLTS
T82 MMLQLGGTVIGSARC
H103 EGRLRAAHNLVKRGI
K107 RAAHNLVKRGITNLC
S130 TGADTFRSEWSDLLS
S133 DTFRSEWSDLLSDLQ
S137 SEWSDLLSDLQKAGK
K141 DLLSDLQKAGKITAE
K144 SDLQKAGKITAEEAT
S198 AITTTAQSHQRTFVL
K272 GAIDRNGKPITSEGV
K280 PITSEGVKDLVVRRL
T304 GHVQRGGTPSAFDRI
T328 VMALLEGTPDTPACV
T331 LLEGTPDTPACVVSL
K360 QVTKDVTKAMDEKRF
K360 QVTKDVTKAMDEKRF
R366 TKAMDEKRFDEAMKL
R366 TKAMDEKRFDEAMKL
K372 KRFDEAMKLRGRsFM
K372 KRFDEAMKLRGRsFM
S377-p AMKLRGRsFMNNWEV
S398 IRPPAPKSGSYTVAV
S400 PPAPKSGSYTVAVMN
T402 APKSGSYTVAVMNVG
K445 DGFEGPAKGQIEEAG
K466 WTGQGGSKLGSKRTL
T472 SKLGSKRTLPKKSFE
K476 SKRTLPKKSFEQISA
S477 KRTLPKKSFEQISAN
Y502 IIGGFEAYTGGLELM
K564 KQSAAGTKRRVFIIE
Y576 IIETMGGYCGYLATM
K615 NVEHLVQKMKTTVKR
K656 KGIFDSRKNVLGHMQ
S667 GHMQQGGSPTPFDRN
K678 FDRNFATKMGAKAMN
K682 FATKMGAKAMNWMAG
Q727 FQPVTELQNQTDFEH
Q727 FQPVTELQNQTDFEH
Y755 ILKILAKYEIDLDTS
S775-p EHISRKRsGEATV__
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