Conserved Protein Domain Family
Pat_PNPLA2

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cd07220: Pat_PNPLA2 
Patatin-like phospholipase domain containing protein 2
PNPLA2 plays a key role in hydrolysis of stored triacylglecerols and is also known as adipose triglyceride lipase (ATGL). Members of this family share a patain domain, initially discovered in potato tubers. ATGL is expressed in white and brown adipose tissue in high mRNA levels. Mutations in PNPLA2 encoding adipose triglyceride lipase (ATGL) leads to neutral lipid storage disease (NLSD) which is characterized by the accumulation of triglycerides in multiple tissues. ATGL mutations are also commonly associated with severe forms of skeletal- and cardio-myopathy. This family includes patatin-like proteins: TTS-2.2 (transport-secretion protein 2.2), PNPLA2 (Patatin-like phospholipase domain-containing protein 2), and iPLA2-zeta (Calcium-independent phospholipase A2) from Homo sapiens.
Statistics
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PSSM-Id: 132859
View PSSM: cd07220
Aligned: 11 rows
Threshold Bit Score: 426.467
Threshold Setting Gi: 47211514
Created: 12-Feb-2009
Updated: 17-Jan-2013
Structure
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Aligned Rows:
 
active sitenucleophile
Feature 1:active site [active site]
Evidence:
  • Comment:Based on the crystal structure of semet patatin (1OXW)
  • Comment:catalytic dyad is formed by Ser and Asp
  • Comment:oxyanion hole is formed by two Gly and one Arg

Sequence Alignment
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Format: Row Display: Color Bits: Type Selection:
                         10        20        30        40        50        60        70        80
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....|
Feature 1                   ## #                            #                                    
gi 74731110    4 REKTWNISFAGCGFLGVYYVGVASCLREHAPFLVanATHIYGASAGALTATALVTGVCLGEAgakfieVSKEARKRFLGP 83
gi 194399018   4 LDSAWNISFAGCGFLGVYHIGVASCLQEHAPFLVanARKVYGASAGALTATALVSGACLGEAgasiirVSKEARKRFLGP 83
gi 118404478   4 LDSPWNISFAGCGFLGIYHIGVASCLKEHTPFLVenARNIYGASAGALTATALVSGACLGEAganiidVSKEARKRFLGP 83
gi 148222401   4 LERGWNLSFAGCGFLGVYHVGVSSCLQERAPQILtrANKIYGASAGALNAAALVCGCSLAQCcsdvlgVAKEARKRNLGP 83
gi 50539736    4 LKEGWNLSFAGCGFLGIYHIGVASCLLEQAPYLIrgATKIYGASAGALTASVLTTEACIEKCcedvinVAKEARKRNLGP 83
gi 47217250    8 LTGGWNLSFAGCGFLGIYHIGVASCLLEKAPYLVkgASKLYGASAGALTASVLASQAALTKCcedvieVAKEARKRNLGP 87
gi 47224528    8 LDSPWNISFAGCGFLGIYHVGVASCLLEQAPFLVhnARHIYGASAGALTATALVTGVCLGETgasiidVAKEARKRFLGP 87
gi 219504781   1 RADKMNISFSGCGFLGMYHVGVASCIMQHAPNLQ--LQKLGGASAGALSAAMLLFGCDLRECtdsvlrLATQARKRVLGP 78
gi 186703000   4 LDSAWNISFAGCGFLGVYHIGVASCLQEHAPFLVanARKVYGASAGALTATALVSGACLGEAgasiirVSKEARKRFLGP 83
gi 47211514    7 LQRDWSLSFAGCGFMGVYYVGVSSCVLERCPRLVrdASAIYGASAGALMAAVLSAGVPLEKScsalmlMAREARSHRLGP 86
gi 9663153    34 REKTWNISFAGCGFLGVYYVGVASCLREHAPFLVanATHIYGASAGALTATALVTGVCLGEAgakfieVSKEARKRFLGP 113
                         90       100       110       120       130       140       150       160
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....|
Feature 1                                                                                        
gi 74731110   84 LHPSFNLVKIIRsfLLKVLPADSHEhASGRLGISLTRVSDGENVIISHFNSKDELIQANVCSGFIPVYCGLIPPs----- 158
gi 194399018  84 LHPSFNLVKIIRlcLSKTVPENGHEvAAGRLGISLTRVSDGENVILSDFHSKEELIQACVCSTFIPVYCGLIPPt----- 158
gi 118404478  84 LHPSFNLVKIIRncLYKTLPEDAHEkATGRLGISLTRVSDGENVLLSQFNTKEELIQACVCSTFIPVYCGLIPPc----- 158
gi 148222401  84 LHPSFNLVKILKkgLYRNLPENAHElASGKLCISLTRVSDGENVLVSEFNSKEELIQALICSAFVPIYCGIIPPs----- 158
gi 50539736   84 LHPTFNIVKVIRggLYRDLPSNAHTlSSGRLCVSLTRVMDGQNVLVSDFSSKEELIQALICSCFIPVYCGLIPPa----- 158
gi 47217250   88 LHPSFNLVKVLKtgLNRDLPSDAHVlASGRLCISLTRVSDGENVLVSDFGSKEELIQALVCSCFIPIYCGLIPPa----- 162
gi 47224528   88 MHPSFNLVKIVRhmMGRTLPDGCHHqASGRLGISLTRVTDGENVLVTHFDDKEELVQACVCSAFIPVYCGIIPPt----- 162
gi 219504781  79 MHPSFRLVKTLRegMRRVFPPNAHEiASGRLCLSVTRVCDGQNVILDQFDTREELIQALICSAFVPVYCGLVPPm----- 153
gi 186703000  84 LHPSFNLVKIIRlcLSKTVPENGHEvAAGRLGISLTRVSDGENVILSDFHSKEELIQACVCSTFIPVYCGLIPPt----- 158
gi 47211514   87 LHPSFNLLQMLKdsLLSSLPEDAHLrVSGRLAVSLTRVPDGKNVLVSDFSSRDELVQALLCSCFVPLYCGVVPPtyrgvv 166
gi 9663153   114 LHPSFNLVKIIRsfLLKVLPADSHEhASGRLGISLTRVSDGENVIISHFNSKDELIQANVCSGFIPVYCGLIPPs----- 188
                        170       180       190       200       210       220       230       240
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*....|
Feature 1                                                    #                                   
gi 74731110  159 -------------------------------------LQGVRYVDGGISDNLPlYELKNTITVSPFSGESDICPQDs-ST 200
gi 194399018 159 -------------------------------------LRGVRYVDGGISDNLPqYELKNTITVSPFSGESDICPRDs-ST 200
gi 118404478 159 -------------------------------------LQGVRYVDGGISDNLPqYELKNTITVSPFSGESDICPRDg-CT 200
gi 148222401 159 -------------------------------------FRGVRYVDGGISNNLPeYDLKNTITVSPFSGESDICPRDtnST 201
gi 50539736  159 -------------------------------------FRGVRYVDGGISDNLPqSELKNTITVSPFSGESDICPKDn-ST 200
gi 47217250  163 -------------------------------------FRGVRYVDGGISDNLPqSELKNTITISPFSGESDICPRDt-ST 204
gi 47224528  163 -------------------------------------LRGVRYVDGGISDNLPqYELKNTITVSPFSGESDICPRDt-ST 204
gi 219504781 154 -------------------------------------FKGTRYVDGGLSDNCP-HLGKNTINVSPFSGEADICPVDklSN 195
gi 186703000 159 -------------------------------------LRGVRYVDGGISDNLPrYELKNTITVSPFSGESDICPRDs-ST 200
gi 47211514  167 sprwkaecpytkgrsysqracvcvcvcvcvrgveppaLSPQHYVDGAVSDNLPrCWTENTVTISAYAGESDLCPPRgnSI 246
gi 9663153   189 -------------------------------------LQGVRYVDGGISDNLPlYELKNTITVSPFSGESDICPQDs-ST 230
                        250       260       270       280       290       300       310
                 ....*....|....*....|....*....|....*....|....*....|....*....|....*....|....*..
Feature 1                                                                                     
gi 74731110  201 NIHELRVTNTSIQFNLRNLYRLSKALFPPEPl-------------------------VLREMCKQGYRDGLRFLQRN 252
gi 194399018 201 NMHELRVTNTSIQFNLRNLYRLSKALFPPEPq-------------------------VLRDMCKQGYRDALHFLKKN 252
gi 118404478 201 NLHELRVTNTSIQFNLSNLYRLSKALFPPEPq-------------------------VLKDMCKQGYRDALHFLKRN 252
gi 148222401 202 NFHEVRLTNTSIQFSLGNLYRLTRALFPPDPt-------------------------VLGEMCQQGYNDALRFLKDN 253
gi 50539736  201 SFHELRFTNTSIQFNLDNAYRLSKALFPPEPk-------------------------VLAEMCQSGYKDALRFLQEN 252
gi 47217250  205 SFHELRFTNTSIQMNLGNMYRLSRALFPPEPk-------------------------VLAEMCQSGYKDALRFLEEN 256
gi 47224528  205 NIHELRFTNTSIQFTLTNLYRVSRALFPPDPv-------------------------VMKVMCKQGYKDALHFLKKN 256
gi 219504781 196 NLLNFSMTNTSIQFTTTNMWRMAIAFFPPEPe-------------------------VLQEICRQGFNDALRFLREN 247
gi 186703000 201 NMHELRVTNTSIQFNLRNLYRLSKALFPPEPq-------------------------VLRDMCKQGYRDALHFLKKN 252
gi 47211514  247 SFHQVRFNNVSIQVNAENMYRVASTFFPPEPqvsrassapagrwrrrqrrvdsrvlqAMAEICHSGYVDALRFLQEN 323
gi 9663153   231 NIHELRVTNTSIQFNLRNLYRLSKALFPPEPl-------------------------VLREMCKQGYRDGLRFLQRN 282

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