4AW6,2YPT,4IL3


Conserved Protein Domain Family
M48A_Zmpste24p_like

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cd07343: M48A_Zmpste24p_like 
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Peptidase M48 subfamily A, a type 1 CaaX endopeptidase
This family contains peptidase family M48 subfamily A which includes a number of well-characterized genes such as those found in humans (ZMPSTE24, also known as farnesylated protein-converting enzyme 1 or FACE-1 or Hs Ste24), Taenia solium metacestode (TsSte24p), Arabidopsis (AtSte24) and yeast (Ste24p). Ste24p contains the zinc metalloprotease motif (HEXXH), likely exposed on the cytoplasmic side. It is thought to be intimately associated with the endoplasmic reticulum (ER), regardless of whether its genes possess the conventional signal motif (KKXX) in the C-terminal. Proteins in this family proteolytically remove the C-terminal three residues of farnesylated proteins. Ste24p is involved in the post-translational processing of prelamin A to mature lamin A, a major component of the nuclear envelope. ZmpSte24 deficiency causes an accumulation of prelamin A leading to lipodystrophy and other disease phenotypes, while mutations in this gene or in that encoding its substrate, prelamin A, result in a series of human inherited diseases known as laminopathies, the most severe of which are Hutchinson Gilford progeria syndrome (HGPS) and restrictive dermopathy (RD) which arise due to unsuccessful maturation of prelamin A. Two forms of mandibuloacral dysplasia, a condition that causes a variety of abnormalities involving bone development, skin pigmentation, and fat distribution, are caused by mutations in two different genes; mutations in the LMNA gene, which normally provides instructions for making lamin A and lamin C, cause mandibuloacral dysplasia with A-type lipodystrophy (MAD-A), and mutations in the ZMPSTE24 gene cause mandibuloacral dysplasia with B-type lipodystrophy (MAD-B). Within cells, these genes are involved in maintaining the structure of the nucleus and may play a role in many cellular processes. Certain HIV protease inhibitors have been shown to inhibit the enzymatic activity of ZMPSTE24, but not enzymes involved in prelamin A processing.
Statistics
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PSSM-Id: 320702
View PSSM: cd07343
Aligned: 371 rows
Threshold Bit Score: 240.461
Threshold Setting Gi: 325948658
Created: 4-Mar-2009
Updated: 18-Aug-2016
Structure
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Program:
Drawing:
Aligned Rows:
 
Zn binding siteactive site
Conserved site includes 4 residues -Click on image for an interactive view with Cn3D
Feature 1:Zn binding site [ion binding site]
Evidence:

Sequence Alignment
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Format: Row Display: Color Bits: Type Selection:
Feature 1                                                                                        
4AW6_A        21 GAVLLFSWTVYLWETFLAQRQRRIYKttthvppelgqimdsetfeksRLYQLDKSTFSFWSGLYSETEGTLILLFggipy 100 human
4IL3_A        17 LIISAFSIAQFSFESYLTYRQYQKLSetklppvled-eiddetfhksRNYSRAKAKFSIFSDIYNLAQKLVFIKYdffpk 95  Saccharomyces m...
CBL87571      25 NIFIVLVIFNFLFTTVLEYLNDKNWNdnipdd---------fkdfydAKSYLKAKNYKISRGKLSSVSSSVSFVFtiaml 95  uncultured Flav...
ELR70029       8 TIVLIIITADFLLEQVLEYINLRSAKddipdd---------iaafydRNEYKRSREYQRTLTRFSFLTATFSFIVsflll 78  Fulvivirga imte...
EON76897       8 YLLIGILSVNFISSKFLNWLNIRRKEngipkt---------megyldTEKLTKANEYQRVNFNFSLVASFFSFGLtilfl 78  Cyclobacteriace...
KGA08568       9 IGILAFVAIDFLIGTLLNYLNEKSNQqplseq---------vsqiydLTGYNKSLEYGTTKYKFGLFTSVVSTIVillai 79  actinobacterium...
GAL87419       8 YIILGILSFDFILEQFFDYLNLKHQKtdlpee---------lkdiysEEEFKKAQDYYKANANFSFLSSTLSFLLiiivi 78  Sporocytophaga ...
WP_039751652  10 TIILFLVMAGFVFDTFLSWLNLRSQPddipds---------vaafydREKYRKSLEYHRHRTNFSFVTSTFSFLLsvaml 80  Hassallia bysso...
KXK31425       8 QIILGIALLSYAFDQLLDYLNLKALRkdipee---------iatfynPEKYNRSLDYQRTQARFSFITAAFGFILsftml 78  Bacteroidetes b...
AHM60012      28 IILISLVALGYLLESALDWLNYTYLKkpylpq---------lsgiltEEKYSETVRYQQERKRFGFITATYSFVLmlvml 98  Flammeovirgacea...
Feature 1                                                                                        
4AW6_A       101 lwrlsg---rfcgyagfgpeYEITQSLVFLLLATLFSALAGLPWSLYNTFVIEEKHGFNqqTLGFFMKDAIKKFVVTQCI 177 human
4IL3_A        96 iwhmavtlsnavlpvrfhmvSTVAQSLCFLGLLSSMSTLVDLPLSYYSHFVLEEKFGFNklTVKLWITDMIKSLTLAYAI 175 Saccharomyces m...
CBL87571      96 yffgfg---flsdiatsfsdSVILQSSLFFMILYLFNFVIGIPFSYYSTFVIEEKFGFNktNLKTFISDKIKGLFISSAL 172 uncultured Flav...
ELR70029      79 itggfg---yidellrpffeNEIYLALSYFGILFLASDLLNTPFEIYQTFVIEEKYGFNktTPKIFIMDKLKGYLLTIAI 155 Fulvivirga imte...
EON76897      79 asgffg---wldaflsqftiHPLWLPALYFAVLYLGSDLMGIPFDYYHTFVIEQRFGFNntSMGTFLSDKLKGYLLSIVI 155 Cyclobacteriace...
KGA08568      80 vlgwfa---wldaslrevirSDLMVTVSFVGILIVLAAITGLPTSYYATFVIEEKFGFNktTKKLFIADNIKQTLLSLAL 156 actinobacterium...
GAL87419      79 ltgtfg---wldkqlsfkfqNPTTLALVFFGILFFVSDIINIPFSLYKTFVIEEKFGFNktTIKTYVLDKLKGYLIGGVL 155 Sporocytophaga ...
WP_039751652  81 yfggfg---lldswlrpvihNDILLALIFFGVLAIASDILTLPFQLYSTFVIEEKYGFNktTISTFIGDKLKGYAVGTIL 157 Hassallia bysso...
KXK31425      79 atggfg---widslvrphfpNEIVLALIFFGVIMIASDLLTLPFQWYSTFVIEEKFGFNktTPKTFIADKIKGYALGAIV 155 Bacteroidetes b...
AHM60012      99 ltggfg---wldeqlrewtqHPILLALLYFGMLMLAADILSLPFQLYSTFRIEESWGFNktSVRTFWLDKLKGYGLGALL 175 Flammeovirgacea...
Feature 1                                                                                        
4AW6_A       178 LLPVSSLLLYIIkiggdyFFIYAWLFTLVVSLVLVTIYADYIAPLFDKFTPLpEGKLKEEIEVMAKSIDFPL-TKVYVVE 256 human
4IL3_A       176 GGPILYLFLKIFdkfptdFLWYIMVFLFVVQILAMTIIPVFIMPLFNKFTPLeDGELKKSIESLADRVGFPL-DKIFVID 254 Saccharomyces m...
CBL87571     173 IVGLTSLAIFIIesfssgYWLWLWLGLSSLMIFLNMFYADLIVPIFNKLTPLeDGELRKKIEAYSNKVGYSLkNIFVIDG 252 uncultured Flav...
ELR70029     156 GGLLIGLLLYLIlnigesFWVYFWVVAALFILFVNMFYTSLVLPLFNKLTPLgEGELKTAIENYSRKVNFPLdNIFVIDG 235 Fulvivirga imte...
EON76897     156 GGSLLLTFLLLVnymgvdFWWQFWLVTGVFMVLVNGLFSSYILPLFNKLTPLeEGELRQAILSYASKAGFPLeNVFVIDG 235 Cyclobacteriace...
KGA08568     157 GLPLIGLIAWIYqelnslFWLVGWLLISVFSLFMFIFGTRIFLPLFNKLKPLpAGELRDEVEAYCQSQGFPLsKLWEMDA 236 actinobacterium...
GAL87419     156 GGLLIWLFIFMVtsigpgFWLYFWIILSVVMLIINMFYTSIFVPLFNKLTPLeAGDLRNSIEEYSKTNNFSLtNIFVIDG 235 Sporocytophaga ...
WP_039751652 158 GGGLISLLIYLIqligpdFWILFSLIAAVLILLINMFYTTIFLPLFNKLTPLeDGELKTKIESFSKTVGFPLdNVFVMDG 237 Hassallia bysso...
KXK31425     156 GGGLLALLIYLIqwigpdFWIWFGMVAALFILIVNMFYTTLILPLFNKLTPLaEGDLKTAITTYAKQVDFPLeNIFVMDG 235 Bacteroidetes b...
AHM60012     176 GGLLLSLLLWLVlelgqnFWLYFWGVLILFMLGANVFYTSLILPLFNKLRPLtAGALKTALETYSQKINFPLkNIYVIDG 255 Flammeovirgacea...
Feature 1                                                                                       #
4AW6_A       257 GSKRssHSNAYFYGFFKn-KRIVLFDTLLEeysvlnkdiqedsgmeprneeegnseeikakvknkkqgcknEEVLAVLGH 335 human
4IL3_A       255 GSKRssHSNAYFTGLPFtsKRIVLFDTLVNsn-------------------------------------stDEITAVLAH 297 Saccharomyces m...
CBL87571     253 SKRSs-KANAFFSGLGPr-KTIALYDTLIKkh-------------------------------------sdEELVSVLAH 293 uncultured Flav...
ELR70029     236 SKRSk-KANAFFSGIGKr-KKIVLYDTLIEnh-------------------------------------stDELVAILAH 276 Fulvivirga imte...
EON76897     236 SKRSs-KANAFFSGFGKr-KKVVLYDTLIAqh-------------------------------------stDELVAVLAH 276 Cyclobacteriace...
KGA08568     237 SKRSt-KLNAFFSGMGKv-KIIGLYDTLIQkl-------------------------------------ttKEVVAVLAH 277 actinobacterium...
GAL87419     236 SKRSs-KANAFFSGIGPk-KKVVLYDTLINnh-------------------------------------skEELVAVLAH 276 Sporocytophaga ...
WP_039751652 238 SKRSn-KANAFFSGIGRk-KKIVLYDTLIAnh-------------------------------------ntDELVAVLAH 278 Hassallia bysso...
KXK31425     236 SKRSa-KANAFFSGIGKk-KKIVLYDTLINnh-------------------------------------ttDELVAVLAH 276 Bacteroidetes b...
AHM60012     256 SKRSs-KANAFFAGLGKq-KKIVLYDTLIEkh-------------------------------------teEEIVAVLAH 296 Flammeovirgacea...
Feature 1           #                                                                            
4AW6_A       336 ELGHWKLGHTVKNIIISQMNSFLCFFLFAVligrkelfaa-------------fgfydsqptlIGLLIIFQfifsPYNEV 402 human
4IL3_A       298 EIGHWQKNHIVNMVIFSQLHTFLIFSLFTSiyrnssfyntfgffveksssgfvdpvitkefpiIIGFMLFNdlltPLECA 377 Saccharomyces m...
CBL87571     294 EVGHFKKKHILLSMIITICQLGVMCYLFEIcmsfemiansl------------gssamnfhigIIAFSFLYs---PIGLI 358 uncultured Flav...
ELR70029     277 EVGHFKKKHIIGSFIFSILQIGIMLFIMSRmiynenlslal------------gaeqmgvhlnLLAFGILYs---PISKV 341 Fulvivirga imte...
EON76897     277 EIGHYKKRHIVTNLVLGILQTGLILFVLAQlifseslslal------------ggsemavhlnLVGFTMLFs---PLSAL 341 Cyclobacteriace...
KGA08568     278 EVGHYKRKHVYTMFLFSNVQTLILFALMGWllgnpnlaral------------gseipsfhlsLISFVLLFs---PLSTI 342 actinobacterium...
GAL87419     277 EIGHFKKKHIITGLILGIIQTGFMLFLLSYfifnknlseal------------gadhlaihinLIAFGILYt---PISHI 341 Sporocytophaga ...
WP_039751652 279 EVGHYKKKHIIFGYILSVIQVVFTLFILSLmvqsenvslal------------ggtqsaihlnLLAFGILFs---PISGL 343 Hassallia bysso...
KXK31425     277 EVGHFKKKHIILTYLISILQVFFVLFVLSTmvfneklsaal------------ggsqqaihlnLIAFTILFs---PISGI 341 Bacteroidetes b...
AHM60012     297 EAGHYKKRHILKGFVLSVLQSGLMLFLLSRfigsetltlalggtpl--ggtpleagllplhlnLIGFGLLYs---PISML 371 Flammeovirgacea...
Feature 1                    #                                             #            
4AW6_A       403 LSFCLTVLSRRFEFQADAFAKKLGka--kdLYSALIKLNKDNLGFPvSDWLFSMWHYSHPPLlERLQALKT 471 human
4IL3_A       378 MQFIMSLISRTHEYQADAYAKKLGyk--qnLCRALIDLQIKNLSTMnVDPLYSSYHYSHPTLaERLTALDY 446 Saccharomyces mikatae
CBL87571     359 IGILMNILSRKNEFEADEYAKLTYdg--naLSQALKKLSVDSLSNLyPHPLYVFVHYSHPPLiKRLNALNK 427 uncultured Flavobacterii...
ELR70029     342 LGIISNVISRKNEFEADAYAGTTYgg--saLAASLKKLSVDNLSNLyPHPAYVFMYYSHPPLlKRLGALKN 410 Fulvivirga imtechensis AK7
EON76897     342 IGVGMNILSRKHEFEADAFAKSTFdg--qpLATALKKLSVNNLTNLnPHPWYVFMNYSHPPLlQRLAKLET 410 Cyclobacteriaceae bacter...
KGA08568     343 LGLIANSFSRFNEYQADQFSIDTYpgarehLHSALKKLSVESLKDLnPHPMYVKVNYSHPPIlERLANLKS 413 actinobacterium acAMD-5
GAL87419     342 TGLLMNIISRKNEYEADRFAAVTYss--iaLQEALKKLSSKNLSNLtPHPAYVFMHYSHPPLlYRLRALKQ 410 Sporocytophaga myxococco...
WP_039751652 344 TGLFMSMYSRKNEFEADAYAKETFsg--paLASALKKLSVDNLSDLyPHPLYVFFHYSHPPLlKRLAVIDQ 412 Hassallia byssoidea
KXK31425     342 TGLLMSMLSRKNEFEADAYAKNTFdg--taLANALKKLSVDTLSNLyPHPWYVFFHYSHPPLlKRLQALEL 410 Bacteroidetes bacterium ...
AHM60012     372 LGLFMNSLSRKHEYQADAYAAQTYga--epLQEALRKLSVLNLSQPlPHPWYVWVHYSHPPLlYRLDALEK 440 Flammeovirgaceae bacteri...

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