Basic Information | Overview of PTM Sites | Experimental PTM Sites | Protein-Protein Interactions | Drug and Disease Associations | Related Literatures
Basic Information
Protein Name :  Pyruvate carboxylase, mitochondrial  

UniProtKB / Swiss-Prot ID :  PYC_HUMAN

Gene Name (Synonyms) : 
PC  

Species :  Homo sapiens (Human). 

Subcellular Localization :  Mitochondrion matrix. 

Protein Function :  Pyruvate carboxylase catalyzes a 2-step reaction, involving the ATP-dependent carboxylation of the covalently attached biotin in the first step and the transfer of the carboxyl group to pyruvate in the second. Catalyzes in a tissue specific manner, the initial reactions of glucose (liver, kidney) and lipid (adipose tissue, liver, brain) synthesis from pyruvate. 

Protein Sequence MLKFRTVHGGLRLLGIRRTSTAPAASPNVRRLEYKPIKKVMVANRGEIAIRVFRACTELGIRTVAIYSEQ...
Predicted Secondary Structure CCEEEEEHHEEEHEEEECCEEECCCCCCCCCCCCCCCEEEEEECCCHHHHHHHHHHHHCCCEEEEECCCH...
Protein Variant
LocationDescription
76H -> L (in dbSNP:rs7104156). VAR_048416
145V -> A (in PC deficiency; mild;dbSNP:rs28940591).
156R -> Q (in PC deficiency). VAR_058957
270R -> W (in PC deficiency). VAR_058958
304Y -> C (in PC deficiency). VAR_058959
451R -> C (in PC deficiency; mild). VAR_015200
583R -> L (in PC deficiency). VAR_058960
610A -> T (in PC deficiency; mild;dbSNP:rs28940589).
631R -> Q (in PC deficiency). VAR_058961
743M -> I (in PC deficiency; mild;dbSNP:rs28940590).
1131Missing (in PC deficiency). VAR_058962
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Overview of Protein Modification Sites with Functional and Structural Information
Accessible Surface Area (ASA)
Pred. Secondary
Real Secondary
Disorder Prediction
Protein Domain
&
Experimental PTM Sites
Predicted PTM Sites
Protein Variant
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Experimental Post-Translational Modification Sites Download
Locations
Modification
Substrate Sites
&
Secondary Structure
Accessible Surface Area (%)
Resource
Reference
Structural Characterization
Orthologous
Protein Cluster
118PhosphotyrosineVHPGYGFLS
EEECCCCCC
14.78Phosphositeplus
Link-
289PhosphothreoninePQLRTRLTS
HHHHHHHHH
38.33HPRD
Link-
293PhosphoserineTRLTSDSVK
HHHHHHHHH
32.26HPRD
Link-
304PhosphotyrosineKQVGYENAG
HHCCCEEEE
13.73HPRD
Link-
304PhosphotyrosineKQVGYENAG
HHCCCEEEE
13.73Phosphositeplus
Link-
309PhosphothreonineENAGTVEFL
EEEEEEEEE
17.91HPRD
Link-
309PhosphothreonineENAGTVEFL
EEEEEEEEE
17.91Phosphositeplus
Link-
741N6-carboxylysine.ILCIKDMAG
EEEECCCEE
40.22UniProtKB
Link
894PhosphotyrosineVKKAYVEAN
HHHHHHHHH
14.01HPRD
Link
894Phosphotyrosine.VKKAYVEAN
HHHHHHHHH
14.01UniProtKB
Link
992N6-acetyllysineQALEKELVD
HHHHHHHHH
58.14Phosphositeplus
Link
1090N6-acetyllysineSILVKDTQA
EEEEECCEE
52.17HPRD
Link
1090N6-acetyllysineSILVKDTQA
EEEEECCEE
52.17Phosphositeplus
Link
1090N6-acetyllysine.SILVKDTQA
EEEEECCEE
52.17UniProtKB
Link
1144N6-biotinyllysine.LSAMKMETV
EEECCEEEE
33.05UniProtKB
Link
1156PhosphothreoninePMEGTVRKV
CCCEEEEEE
12.11Phosphositeplus
Link
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Protein-Protein Interactions
      Interacting Protein      
Interaction type
Source ID
      Resource      
      Pubmed ID      
Domain-Domain Interactions
MDHM_HUMANin vitroHPRD:02032HPRD8349677
AATM_HUMANin vitroHPRD:02032HPRD8349677
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Disease Reference
Kegg disease
OMIM disease
266150Pyruvate carboxylase deficiency (PC deficiency)
Drug Reference
DrugBank
DB00121Biotin
DB00119Pyruvic acid
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Related Literatures of Post-Translational Modification
Acetylation
ReferencePubMed
"Lysine acetylation targets protein complexes and co-regulates majorcellular functions.";
Choudhary C., Kumar C., Gnad F., Nielsen M.L., Rehman M., Walther T.,Olsen J.V., Mann M.;
Science 325:834-840(2009).
Cited for: ACETYLATION [LARGE SCALE ANALYSIS] AT LYS-1090, AND MASS SPECTROMETRY.
Phosphorylation
ReferencePubMed
"Improved titanium dioxide enrichment of phosphopeptides from HeLacells and high confident phosphopeptide identification by cross-validation of MS/MS and MS/MS/MS spectra.";
Yu L.-R., Zhu Z., Chan K.C., Issaq H.J., Dimitrov D.S., Veenstra T.D.;
J. Proteome Res. 6:4150-4162(2007).
Cited for: PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT TYR-894, AND MASSSPECTROMETRY.
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Basic Information | Overview of PTM Sites | Experimental PTM Sites | Protein-Protein Interactions | Drug and Disease Associations | Related Literatures