Alpha-1-antitrypsin

NameAlpha-1-antitrypsin
Synonyms
  • AAT
  • Alpha-1 protease inhibitor
  • Alpha-1-antiproteinase
  • PI
  • Serpin A1
Gene NameSERPINA1
OrganismHuman
Amino acid sequence
>lcl|BSEQ0001896|Alpha-1-antitrypsin
MPSSVSWGILLLAGLCCLVPVSLAEDPQGDAAQKTDTSHHDQDHPTFNKITPNLAEFAFS
LYRQLAHQSNSTNIFFSPVSIATAFAMLSLGTKADTHDEILEGLNFNLTEIPEAQIHEGF
QELLRTLNQPDSQLQLTTGNGLFLSEGLKLVDKFLEDVKKLYHSEAFTVNFGDTEEAKKQ
INDYVEKGTQGKIVDLVKELDRDTVFALVNYIFFKGKWERPFEVKDTEEEDFHVDQVTTV
KVPMMKRLGMFNIQHCKKLSSWVLLMKYLGNATAIFFLPDEGKLQHLENELTHDIITKFL
ENEDRRSASLHLPKLSITGTYDLKSVLGQLGITKVFSNGADLSGVTEEAPLKLSKAVHKA
VLTIDEKGTEAAGAMFLEAIPMSIPPEVKFNKPFVFLMIEQNTKSPLFMGKVVNPTQK
Number of residues418
Molecular Weight46736.195
Theoretical pI5.31
GO Classification
Functions
  • protease binding
  • glycoprotein binding
  • identical protein binding
  • serine-type endopeptidase inhibitor activity
Processes
  • cellular protein metabolic process
  • COPII vesicle coating
  • ER to Golgi vesicle-mediated transport
  • blood coagulation
  • membrane organization
  • platelet degranulation
  • post-translational protein modification
  • protein N-linked glycosylation via asparagine
  • acute-phase response
  • platelet activation
  • negative regulation of endopeptidase activity
Components
  • extracellular exosome
  • proteinaceous extracellular matrix
  • endoplasmic reticulum lumen
  • endoplasmic reticulum-Golgi intermediate compartment membrane
  • ER to Golgi transport vesicle
  • Golgi membrane
  • platelet alpha granule lumen
  • extracellular region
  • endoplasmic reticulum
  • extracellular space
  • Golgi apparatus
General FunctionSerine-type endopeptidase inhibitor activity
Specific FunctionInhibitor of serine proteases. Its primary target is elastase, but it also has a moderate affinity for plasmin and thrombin. Irreversibly inhibits trypsin, chymotrypsin and plasminogen activator. The aberrant form inhibits insulin-induced NO synthesis in platelets, decreases coagulation time and has proteolytic activity against insulin and plasmin.Short peptide from AAT: reversible chymotrypsin inhibitor. It also inhibits elastase, but not trypsin. Its major physiological function is the protection of the lower respiratory tract against proteolytic destruction by human leukocyte elastase (HLE).
Pfam Domain Function
Transmembrane RegionsNot Available
GenBank Protein ID177829
UniProtKB IDP01009
UniProtKB Entry NameA1AT_HUMAN
Cellular LocationSecreted
Gene sequence
>lcl|BSEQ0020495|Alpha-1-antitrypsin (SERPINA1)
ATGCCGTCTTCTGTCTCGTGGGGCATCCTCCTGCTGGCAGGCCTGTGCTGCCTGGTCCCT
GTCTCCCTGGCTGAGGATCCCCAGGGAGATGCTGCCCAGAAGACAGATACATCCCACCAT
GATCAGGATCACCCAACCTTCAACAAGATCACCCCCAACCTGGCTGAGTTCGCCTTCAGC
CTATACCGCCAGCTGGCACACCAGTCCAACAGCACCAATATCTTCTTCTCCCCAGTGAGC
ATCGCTACAGCCTTTGCAATGCTCTCCCTGGGGACCAAGGCTGACACTCACGATGAAATC
CTGGAGGGCCTGAATTTCAACCTCACGGAGATTCCGGAGGCTCAGATCCATGAAGGCTTC
CAGGAACTCCTCCGTACCCTCAACCAGCCAGACAGCCAGCTCCAGCTGACCACCGGCAAT
GGCCTGTTCCTCAGCGAGGGCCTGAAGCTAGTGGATAAGTTTTTGGAGGATGTTAAAAAG
TTGTACCACTCAGAAGCCTTCACTGTCAACTTCGGGGACACCGAAGAGGCCAAGAAACAG
ATCAACGATTACGTGGAGAAGGGTACTCAAGGGAAAATTGTGGATTTGGTCAAGGAGCTT
GACAGAGACACAGTTTTTGCTCTGGTGAATTACATCTTCTTTAAAGGCAAATGGGAGAGA
CCCTTTGAAGTCAAGGACACCGAGGAAGAGGACTTCCACGTGGACCAGGTGACCACCGTG
AAGGTGCCTATGATGAAGCGTTTAGGCATGTTTAACATCCAGCACTGTAAGAAGCTGTCC
AGCTGGGTGCTGCTGATGAAATACCTGGGCAATGCCACCGCCATCTTCTTCCTGCCTGAT
GAGGGGAAACTACAGCACCTGGAAAATGAACTCACCCACGATATCATCACCAAGTTCCTG
GAAAATGAAGACAGAAGGTCTGCCAGCTTACATTTACCCAAACTGTCCATTACTGGAACC
TATGATCTGAAGAGCGTCCTGGGTCAACTGGGCATCACTAAGGTCTTCAGCAATGGGGCT
GACCTCTCCGGGGTCACAGAGGAGGCACCCCTGAAGCTCTCCAAGGCCGTGCATAAGGCT
GTGCTGACCATCGACGAGAAAGGGACTGAAGCTGCTGGGGCCATGTTTTTAGAGGCCATA
CCCATGTCTATCCCCCCCGAGGTCAAGTTCAACAAACCCTTTGTCTTCTTAATGATTGAA
CAAAATACCAAGTCTCCCCTCTTCATGGGAAAAGTGGTGAATCCCACCCAAAAATAA
GenBank Gene IDK01396
GeneCard IDNot Available
GenAtlas IDSERPINA1
HGNC IDHGNC:8941
Chromosome Location14
Locus14q32.1
References
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