1QLP,1OPH,1HP7,1IZ2,1OO8,3CWM,3T1P


Conserved Protein Domain Family
serpinA1_A1AT

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cd02056: serpinA1_A1AT 
Click on image for an interactive view with Cn3D
serpin family A member 1, alpha-1-antitrypsin
Alpha-1-antitrypsin (also called A1AT, A1A, AAT, alpha1-proteinase inhibitor/A1PI, alpha1-antiproteinase/A1AP, proteinase inhibitor/PI, and serum trypsin inhibitor) is a protease inhibitor that belongs to the serpin superfamily. It is encoded in humans by the SERPINA1 gene. When the blood contains inadequate amounts of A1AT or functionally defective A1AT (such as in alpha-1 antitrypsin deficiency), neutrophil elastase is excessively free to break down elastin, degrading the elasticity of the lungs, which results in respiratory complications, such as chronic obstructive pulmonary disease. Normally, A1AT leaves its site of origin, the liver, and joins the systemic circulation; defective A1AT fails to do so, building up in the liver, which results in cirrhosis. This family contains other A1AT-like members of clade A of the serpin superfamily. In general, SERine Proteinase INhibitors (serpins) exhibit conformational polymorphism shifting from native to cleaved, latent, delta, or polymorphic forms. Many serpins, such as antitrypsin and antichymotrypsin, function as serine protease inhibitors which regulate blood coagulation cascades. Non-inhibitory serpins perform many diverse functions such as chaperoning proteins or transporting hormones. Serpins are of medical interest because mutants have been associated with blood clotting disorders, emphysema, cirrhosis, and dementia. A classification based on evolutionary relatedness has resulted in the assignment of serpins to 16 clades designated A-P along with some orphans.
Statistics
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PSSM-Id: 381012
Aligned: 32 rows
Threshold Bit Score: 715.72
Created: 11-Jul-2017
Updated: 2-Oct-2020
Structure
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Program:
Drawing:
Aligned Rows:
 
reactive centertrypsin bindingtrimer
Conserved site includes 26 residues -Click on image for an interactive view with Cn3D
Feature 1:reactive center loop (RCL)
Evidence:
  • Comment:depending on the conformational state, the RC loop is surface accessible in the active form or buried and inserted as the central beta strand in the inactive form.
  • Structure:1QLP_A; Human alpha-1-antitrypsin active conformation-loop out; P1-P1' cleavage site: Met-Ser.
  • Structure:1IZ2_A: human alpha-1-antitrypsin, latent-uncleaved and inserted.
  • Citation:PMID 9466920

Sequence Alignment
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Format: Row Display: Color Bits: Type Selection:
Feature 1                                                                                     
1QLP_A     26 ITPNLAEFAFSLYRQLAHQSNsTNIFFSPVSIATAFAMLSLGTKaDTHDEILEGLNFNltEIPEAQIHEGFQELLRTLNQ 105 human
P23035     45 IAPSLAEFALSLYREVAHESNtTNIFFSPVSIALAFAMLSLGAKgDTHTQVLEGLKFNltETAEAQIHDGFRHLLHTVNR 124 rabbit
P26595     43 DTPYNLELSISLYRELGHKSTtSNIFFSQVSIATAFAMLSLGEKgDTHTQILEGLQFNltQTSEADIHKAFQHLLQTLNR 122 Ryukyu mouse
P07758     44 IATNLGDFAISLYRELVHQSNtSNIFFSPVSIATAFAMLSLGSKgDTHTQILEGLQFNltQTSEADIHKSFQHLLQTLNR 123 house mouse
Q00897     44 IATNLGDFALRLYRELVHQSNtSNIFFSPVSIATAFAMLSLGSKgDTHTQILEGLQFNltQTSEADIHKSFQHLLQTLNR 123 house mouse
Q00898     44 IATNLGDFAISLYRELVHQSNtSNIFFSPVSIATAFAMLSLGSKgDTHTQILEGLQFNltQTSEADIHNSFQHLLQTLNR 123 house mouse
Q63969     44 MATNLGDFAFSLYRELVHQSNtSNIFFSPVSIATAFALLSLGSKgDTQTQILEGLQFNltQTSEADIHKVFQHLLQTLNR 123 spiny mouse
P22599     44 IATNLGDFAISLYRELVHQSNtSNIFFSPVSIATAFAMLSLGSKgDTHTQILEGLQFNltQTSEADIHKSFQHLLQTLNR 123 house mouse
Q00896     44 IATNLGDFAISLYRELVHQSNtSNIFFSPVSIATAFAMLSLGSKgDTHTQILEGLQFNltQTSEADIHKSFQHLLQTLNR 123 house mouse
P22325     37 IPRSLAHFAHSMYRVLTQQSNtSNIFFSPVSIATALAMVSVGAKgDTHTQILRGLEFNltEIAEADIHNGFQNLLHTLNR 116 domestic guinea pig
Feature 1                                                                                     
1QLP_A    106 pdsqLQLTTGNGLFLSEGLKLVDKFLEDVKKLYhseAFTVNFGDtEEAKKQINDYVEKGTQGKIVDLVkeldRDTVFALV 185 human
P23035    125 pdseLQLAARNALVVHENLKLQHKFLEDAKNLYqseAFLVDFRDpEQAKTKINSHVEKGTRGKIVDLVqeldARTLLALV 204 rabbit
P26595    123 pdseLQLSTGNGSLLNNDLKLVEKFLEEAKNNYhseVFSVNFAEsEEAKKVINDFVEKGTQGKIAEAVkdpdEDTVFALA 202 Ryukyu mouse
P07758    124 pdseLQLSTGNGLFVNNDLKLVEKFLEEAKNHYqaeVFSVNFAEsEEAKKVINDFVEKGTQGKIAEAVkkldQDTVFALA 203 house mouse
Q00897    124 pdseLQLSTGNGLFVNNDLKLVEKFLEEAKNHYqaeVFSVNFAEsEEAKKVINDFVEKGTQGKIVEAVkkldQDTVFALA 203 house mouse
Q00898    124 pdseLQLSTGNGLFVNNDLKLVEKFLEEAKNHYqaeVFSVNFAEsEEAKKVINDFVEKGTQGKIVEAVkkleQDTVFVLA 203 house mouse
Q63969    124 pdseLQLSTGNGLFVNNDLKLVEKFLEEAKNHYqseVFSVNFAKsEEARKMINDFVEKGTQGKIVDAVkdldEDTVFALA 203 spiny mouse
P22599    124 pdseLQLSTGNGLFVNNDLKLVEKFLEEAKNHYqaeVFSVNFAEsEEAKKVINDFVEKGTQGKIVEAVkeldQDTVFALA 203 house mouse
Q00896    124 pdseLQLSTGNGLFVNNDLKLVEKFLEEAKNHYqaeVFSVNFAEsEEAKKVINDFVEKGTQGKIVEAVkkldQDTVFALA 203 house mouse
P22325    117 phseHQLTTGNGLFLDQKLKLKEKFSEDVKTLYhaeAFPTNFSNpKEAEKQINAYVEKGTQGKIVDLVkdlsADTVLALV 196 domestic guinea pig
Feature 1                                                                                     
1QLP_A    186 NYIFFKGKWERPFEVKDTEEEDFHVDQVTTVKVPMMKRLGMFNIQHCKKLSSWVLLMKYLGNATAIFFLPDEGKLQHLEN 265 human
P23035    205 NYVFFKGKWEKPFEPENTKEEDFHVNATTTVRVPMMSRLGRYDLFHCSTLASTVLRMDYKGNATALFLLPDEGKLQHLED 284 rabbit
P26595    203 NYILFKGKWKKPFDPKHTEEAEFHVDTVTTVKVPMMTLTGMLDVHHCSTLSSWVLLMDYLGNRTAVFLLPDDGKMQHLEQ 282 Ryukyu mouse
P07758    204 NYILFKGKWKKPFDPENTEEAEFHVDESTTVKVPMMTLSGMLHVHHCSTLSSWVLLMDYAGNATAVFLLPDDGKMQHLEQ 283 house mouse
Q00897    204 NYILFKGKWKQPFDPENTEEAEFHVDESTTVKVPMMTLSGMLDVHHCSMLSSWVLLMDYAGNTTAVFLLPDDGKMQHLEQ 283 house mouse
Q00898    204 NYILFKGKWKKPFDPENTKQAEFHVDESTTVKVPMMTLSGMLDVHHCSTLSSWVLLMDYAGNATAVFLLPDDGKMQHLEQ 283 house mouse
Q63969    204 NYIFFQGKWKTPFDPEHTTEADFHVNESTTVRVPMMNLMRMLDVHYCSTLSSWVLMMDYLGNATAVFLLPDDGKMQHLEQ 283 spiny mouse
P22599    204 NYILFKGKWKKPFDPENTEEAEFHVDKSTTVKVPMMMLSGMLDVHHCSILSSWVLLMDYAGNASAVFLLPEDGKMQHLEQ 283 house mouse
Q00896    204 NYILFKGKWKKPFDPENTEEAEFHVDESTTVKVPMMTLSGMLDVHHCSTLSSWVLLMDYAGNATAVFLLPDDGKMQHLEQ 283 house mouse
P22325    197 NYIFFRGKWEKPFDVKHTTQEDFHVDTSTTVKVPMMKREGKYKAFHCSTIQSWVLLLDYEGNVTALFLLPEEGKMQHLEE 276 domestic guinea pig
Feature 1                                                                                    #
1QLP_A    266 ELTHDIITKFLENEDRRSASLHLPKLSITGTYDLKSVLGQlGITKVFSNGADLSGvte-eaPLKLSKAVHKAVLTIDEKG 344 human
P23035    285 TLTTELITKFLAKSSLRSVTVHFPKLSISGTYDLKPLLGKlGITQVFSDNADLSGite-qePLKASQALHKAVLTIDERG 363 rabbit
P26595    283 TLNKELISKFLLNRHRRLAQVHLPRLSLSGNYTLNTLMSHlGITRIFNNGADLSGiteenaPLKLSKAADKAVLTMDETG 362 Ryukyu mouse
P07758    284 TLSKELISKFLLNRRRRLAQIHFPRLSISGEYNLKTLMSPlGITRIFNNGADLSGiteenaPLKLSQAVHKAVLTIDETG 363 house mouse
Q00897    284 TLNKELISQFLLNRRRSDAQIHIPRLSISGNYNLKTLMSPlGITRIFNNGADLSGiteenaPLKLSKAVHKAVLTIDETG 363 house mouse
Q00898    284 TLNKELISKFLLNRRRRLAQIHIPRLSISGNYNLETLMSPlGITRIFNSGADLSGiteenaPLKLSQAVHKAVLTIDETG 363 house mouse
Q63969    284 TLNKELISKFLLNRHRSLAEIHFPRLSISGSYNLKALMAPlGITRVFNNGADLSGiteenaPLRLSKAVHKAVLTIDERG 363 spiny mouse
P22599    284 TLNKELISKILLNRRRRLVQIHIPRLSISGDYNLKTLMSPlGITRIFNNGADLSGiteenaPLKLSKAVHKAVLTIDETG 363 house mouse
Q00896    284 TLSKELISKFLLNRRRRLAQIHFPRLSISGEYNLKTLMSPlGITRIFNNGADLSGiteenaPLKLSQAVHKAVLTIDETG 363 house mouse
P22325    277 TLTPELIFKFARKTERMFANVHLPKLSISGTYDLKEVLGHlGITNVFSDAADLSGvte-diPLKISKGLHKALLTIDEKG 355 domestic guinea pig
Feature 1     #########################                        
1QLP_A    345 TEAAGAMFLEAIPMSIPPEVKFNKPFVFLMIEQNTKSPLFMGKVVNPTQ 393 human
P23035    364 TEAAGATYMEIIPMSLPDSITLDRPFLFVIYSHEIKSPLFVGKVVDPTQ 412 rabbit
P26595    363 TEAAAATVLQAVPMSMPPILNFNKPFIFIIVEEHTQSPLFVGKVVDPTR 411 Ryukyu mouse
P07758    364 TEAAAVTVLQMVPMSMPPILRFDHPFLFIIFEEHTQSPIFLGKVVDPTH 412 house mouse
Q00897    364 TEAAAATVLQVATYSMPPIVRFDHPFLFIIFEEHTQSPIFVGKVVDPTH 412 house mouse
Q00898    364 TEAAAATVLQGGFLSMPPILHFNRPFLFIIFEEHSQSPLFVGKVVDPTH 412 house mouse
Q63969    364 TEAAATTIVEAVFMSLPPILHFNHPFVFTIVETHTQTPLFVGKVVDPTR 412 spiny mouse
P22599    364 TEAAAATVFEAVPMSMPPILRFDHPFLFIIFEEHTQSPIFVGKVVDPTH 412 house mouse
Q00896    364 TEAAAVTVLLAVPYSMPPILRFDHPFLFIIFEEHTQSPLFVGKVVDPTH 412 house mouse
P22325    356 TEAAGATMMEFMPMSLPEDLSFNKPFLFLIIDHSTDTPLFVGKVMDPTK 404 domestic guinea pig

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