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
Aligned: 11 rows
Threshold Bit Score: 426.467
Created: 12-Feb-2009
Updated: 2-Oct-2020
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:
Feature 1                   ## #                            #                                    
Q96AD5         4 REKTWNISFAGCGFLGVYYVGVASCLREHAPFLVanATHIYGASAGALTATALVTGVCLGEAgakfieVSKEARKRFLGP 83  human
ACF60601       4 LDSAWNISFAGCGFLGVYHIGVASCLQEHAPFLVanARKVYGASAGALTATALVSGACLGEAgasiirVSKEARKRFLGP 83  chicken
NP_001072695   4 LDSPWNISFAGCGFLGIYHIGVASCLKEHTPFLVenARNIYGASAGALTATALVSGACLGEAganiidVSKEARKRFLGP 83  western clawed ...
NP_001087449   4 LERGWNLSFAGCGFLGVYHVGVSSCLQERAPQILtrANKIYGASAGALNAAALVCGCSLAQCcsdvlgVAKEARKRNLGP 83  African clawed ...
NP_001002338   4 LKEGWNLSFAGCGFLGIYHIGVASCLLEQAPYLIrgATKIYGASAGALTASVLTTEACIEKCcedvinVAKEARKRNLGP 83  zebrafish
CAF96773       8 LTGGWNLSFAGCGFLGIYHIGVASCLLEKAPYLVkgASKLYGASAGALTASVLASQAALTKCcedvieVAKEARKRNLGP 87  spotted green p...
CAG08778       8 LDSPWNISFAGCGFLGIYHVGVASCLLEQAPFLVhnARHIYGASAGALTATALVTGVCLGETgasiidVAKEARKRFLGP 87  spotted green p...
XP_002248596   1 RADKMNISFSGCGFLGMYHVGVASCIMQHAPNLQ--LQKLGGASAGALSAAMLLFGCDLRECtdsvlrLATQARKRVLGP 78  Florida lancelet
ACC91734       4 LDSAWNISFAGCGFLGVYHIGVASCLQEHAPFLVanARKVYGASAGALTATALVSGACLGEAgasiirVSKEARKRFLGP 83  chicken
CAF90310       7 LQRDWSLSFAGCGFMGVYYVGVSSCVLERCPRLVrdASAIYGASAGALMAAVLSAGVPLEKScsalmlMAREARSHRLGP 86  spotted green p...
CAC01132      34 REKTWNISFAGCGFLGVYYVGVASCLREHAPFLVanATHIYGASAGALTATALVTGVCLGEAgakfieVSKEARKRFLGP 113 human
Feature 1                                                                                        
Q96AD5        84 LHPSFNLVKIIRsfLLKVLPADSHEhASGRLGISLTRVSDGENVIISHFNSKDELIQANVCSGFIPVYCGLIPPs----- 158 human
ACF60601      84 LHPSFNLVKIIRlcLSKTVPENGHEvAAGRLGISLTRVSDGENVILSDFHSKEELIQACVCSTFIPVYCGLIPPt----- 158 chicken
NP_001072695  84 LHPSFNLVKIIRncLYKTLPEDAHEkATGRLGISLTRVSDGENVLLSQFNTKEELIQACVCSTFIPVYCGLIPPc----- 158 western clawed ...
NP_001087449  84 LHPSFNLVKILKkgLYRNLPENAHElASGKLCISLTRVSDGENVLVSEFNSKEELIQALICSAFVPIYCGIIPPs----- 158 African clawed ...
NP_001002338  84 LHPTFNIVKVIRggLYRDLPSNAHTlSSGRLCVSLTRVMDGQNVLVSDFSSKEELIQALICSCFIPVYCGLIPPa----- 158 zebrafish
CAF96773      88 LHPSFNLVKVLKtgLNRDLPSDAHVlASGRLCISLTRVSDGENVLVSDFGSKEELIQALVCSCFIPIYCGLIPPa----- 162 spotted green p...
CAG08778      88 MHPSFNLVKIVRhmMGRTLPDGCHHqASGRLGISLTRVTDGENVLVTHFDDKEELVQACVCSAFIPVYCGIIPPt----- 162 spotted green p...
XP_002248596  79 MHPSFRLVKTLRegMRRVFPPNAHEiASGRLCLSVTRVCDGQNVILDQFDTREELIQALICSAFVPVYCGLVPPm----- 153 Florida lancelet
ACC91734      84 LHPSFNLVKIIRlcLSKTVPENGHEvAAGRLGISLTRVSDGENVILSDFHSKEELIQACVCSTFIPVYCGLIPPt----- 158 chicken
CAF90310      87 LHPSFNLLQMLKdsLLSSLPEDAHLrVSGRLAVSLTRVPDGKNVLVSDFSSRDELVQALLCSCFVPLYCGVVPPtyrgvv 166 spotted green p...
CAC01132     114 LHPSFNLVKIIRsfLLKVLPADSHEhASGRLGISLTRVSDGENVIISHFNSKDELIQANVCSGFIPVYCGLIPPs----- 188 human
Feature 1                                                    #                                   
Q96AD5       159 -------------------------------------LQGVRYVDGGISDNLPlYELKNTITVSPFSGESDICPQDs-ST 200 human
ACF60601     159 -------------------------------------LRGVRYVDGGISDNLPqYELKNTITVSPFSGESDICPRDs-ST 200 chicken
NP_001072695 159 -------------------------------------LQGVRYVDGGISDNLPqYELKNTITVSPFSGESDICPRDg-CT 200 western clawed ...
NP_001087449 159 -------------------------------------FRGVRYVDGGISNNLPeYDLKNTITVSPFSGESDICPRDtnST 201 African clawed ...
NP_001002338 159 -------------------------------------FRGVRYVDGGISDNLPqSELKNTITVSPFSGESDICPKDn-ST 200 zebrafish
CAF96773     163 -------------------------------------FRGVRYVDGGISDNLPqSELKNTITISPFSGESDICPRDt-ST 204 spotted green p...
CAG08778     163 -------------------------------------LRGVRYVDGGISDNLPqYELKNTITVSPFSGESDICPRDt-ST 204 spotted green p...
XP_002248596 154 -------------------------------------FKGTRYVDGGLSDNCP-HLGKNTINVSPFSGEADICPVDklSN 195 Florida lancelet
ACC91734     159 -------------------------------------LRGVRYVDGGISDNLPrYELKNTITVSPFSGESDICPRDs-ST 200 chicken
CAF90310     167 sprwkaecpytkgrsysqracvcvcvcvcvrgveppaLSPQHYVDGAVSDNLPrCWTENTVTISAYAGESDLCPPRgnSI 246 spotted green p...
CAC01132     189 -------------------------------------LQGVRYVDGGISDNLPlYELKNTITVSPFSGESDICPQDs-ST 230 human
Feature 1                                                                                     
Q96AD5       201 NIHELRVTNTSIQFNLRNLYRLSKALFPPEPl-------------------------VLREMCKQGYRDGLRFLQRN 252 human
ACF60601     201 NMHELRVTNTSIQFNLRNLYRLSKALFPPEPq-------------------------VLRDMCKQGYRDALHFLKKN 252 chicken
NP_001072695 201 NLHELRVTNTSIQFNLSNLYRLSKALFPPEPq-------------------------VLKDMCKQGYRDALHFLKRN 252 western clawed frog
NP_001087449 202 NFHEVRLTNTSIQFSLGNLYRLTRALFPPDPt-------------------------VLGEMCQQGYNDALRFLKDN 253 African clawed frog
NP_001002338 201 SFHELRFTNTSIQFNLDNAYRLSKALFPPEPk-------------------------VLAEMCQSGYKDALRFLQEN 252 zebrafish
CAF96773     205 SFHELRFTNTSIQMNLGNMYRLSRALFPPEPk-------------------------VLAEMCQSGYKDALRFLEEN 256 spotted green puff...
CAG08778     205 NIHELRFTNTSIQFTLTNLYRVSRALFPPDPv-------------------------VMKVMCKQGYKDALHFLKKN 256 spotted green puff...
XP_002248596 196 NLLNFSMTNTSIQFTTTNMWRMAIAFFPPEPe-------------------------VLQEICRQGFNDALRFLREN 247 Florida lancelet
ACC91734     201 NMHELRVTNTSIQFNLRNLYRLSKALFPPEPq-------------------------VLRDMCKQGYRDALHFLKKN 252 chicken
CAF90310     247 SFHQVRFNNVSIQVNAENMYRVASTFFPPEPqvsrassapagrwrrrqrrvdsrvlqAMAEICHSGYVDALRFLQEN 323 spotted green puff...
CAC01132     231 NIHELRVTNTSIQFNLRNLYRLSKALFPPEPl-------------------------VLREMCKQGYRDGLRFLQRN 282 human

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