Alpha-1-Antitrypsin (a1AT)

[Edit]

SERPINA1; SPAAT; A1-AT; PI; Serpin Peptidase Inhibitor,Clade A(Alpha-1 Antiproteinase/AntiTrypsin)Member 1; Alpha-1 protease inhibitor; Short peptide from AAT; Serpin A1

Alpha-1-Antitrypsin (a1AT)
  • alpha1-antitrypsin, an acute phase protein, is the prototypic member of the serpin super family and a major inhibitor of serine proteases. As an acute phase protein, alpha 1-antitrypsin is thought to play an important role in limiting host tissue injury at sites of inflammation.

  • A1AT is a 52-kDa serpin (serine protease inhibitor), and, in medicine, it is considered the most prominent serpin; the terms alpha 1-antitrypsin and protease inhibitor (Pi) are often used interchangeably.Like all serine protease inhibitors, A1AT has a characteristic secondary structure of beta sheets and alpha helices. Mutations in these areas can lead to non-functional proteins that can polymerise and accumulate in the liver (infantile hepatic cirrhosis).

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPB697Hu01 Recombinant Alpha-1-Antitrypsin (a1AT) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAB697Hu01 Polyclonal Antibody to Alpha-1-Antitrypsin (a1AT) WB; IHC; ICC; IP.
MAB697Hu22 Monoclonal Antibody to Alpha-1-Antitrypsin (a1AT) WB; IHC; ICC; IP.
LAB697Hu71 Biotin-Linked Polyclonal Antibody to Alpha-1-Antitrypsin (a1AT) WB; IHC; ICC.
MAB697Hu21 Monoclonal Antibody to Alpha-1-Antitrypsin (a1AT) WB; IHC; ICC; IP.
Assay Kits SEB697Hu ELISA Kit for Alpha-1-Antitrypsin (a1AT) Enzyme-linked immunosorbent assay for Antigen Detection.
SCB697Hu CLIA Kit for Alpha-1-Antitrypsin (a1AT) Chemiluminescent immunoassay for Antigen Detection.

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPB697Mu01 Recombinant Alpha-1-Antitrypsin (a1AT) Positive Control; Immunogen; SDS-PAGE; WB.
RPB697Mu02 Recombinant Alpha-1-Antitrypsin (a1AT) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAB697Mu01 Polyclonal Antibody to Alpha-1-Antitrypsin (a1AT) WB; IHC; ICC; IP.
LAB697Mu71 Biotin-Linked Polyclonal Antibody to Alpha-1-Antitrypsin (a1AT) WB; IHC; ICC.
LAB697Mu81 FITC-Linked Polyclonal Antibody to Alpha-1-Antitrypsin (a1AT) WB; IHC; ICC; IF.
PAB697Mu02 Polyclonal Antibody to Alpha-1-Antitrypsin (a1AT) WB; IHC; ICC; IP.
Assay Kits SEB697Mu ELISA Kit for Alpha-1-Antitrypsin (a1AT) Enzyme-linked immunosorbent assay for Antigen Detection.

Organism species: Rattus norvegicus (Rat)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPB697Ra01 Recombinant Alpha-1-Antitrypsin (a1AT) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAB697Ra01 Polyclonal Antibody to Alpha-1-Antitrypsin (a1AT) WB; IHC; ICC; IP.
LAB697Ra71 Biotin-Linked Polyclonal Antibody to Alpha-1-Antitrypsin (a1AT) WB; IHC; ICC.
MAB697Ra21 Monoclonal Antibody to Alpha-1-Antitrypsin (a1AT) WB; IHC; ICC; IP.
Assay Kits SEB697Ra ELISA Kit for Alpha-1-Antitrypsin (a1AT) Enzyme-linked immunosorbent assay for Antigen Detection.

Organism species: Oryctolagus cuniculus (Rabbit)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Alpha-1-Antitrypsin (a1AT) Recombinant Protein Customized Service Offer
Antibodies PAB697Rb51 Polyclonal Antibody to Alpha-1-Antitrypsin (a1AT) WB; IHC; ICC; IP.
Assay Kits n/a CLIA Kit for Alpha-1-Antitrypsin (a1AT) CLIA Kit Customized Service Offer
n/a ELISA Kit for Alpha-1-Antitrypsin (a1AT) ELISA Kit Customized Service Offer

Organism species: Canis familiaris; Canine (Dog)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Alpha-1-Antitrypsin (a1AT) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Alpha-1-Antitrypsin (a1AT) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Alpha-1-Antitrypsin (a1AT) Polyclonal Antibody Customized Service Offer
Assay Kits CEB697Ca ELISA Kit for Alpha-1-Antitrypsin (a1AT) Enzyme-linked immunosorbent assay for Antigen Detection.

Organism species: Sus scrofa; Porcine (Pig)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Alpha-1-Antitrypsin (a1AT) Recombinant Protein Customized Service Offer
Antibodies PAB697Po01 Polyclonal Antibody to Alpha-1-Antitrypsin (a1AT) WB; IHC; ICC; IP.
Assay Kits n/a CLIA Kit for Alpha-1-Antitrypsin (a1AT) CLIA Kit Customized Service Offer
n/a ELISA Kit for Alpha-1-Antitrypsin (a1AT) ELISA Kit Customized Service Offer

Organism species: Chicken (Gallus)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Alpha-1-Antitrypsin (a1AT) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Alpha-1-Antitrypsin (a1AT) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Alpha-1-Antitrypsin (a1AT) Polyclonal Antibody Customized Service Offer
Assay Kits SEB697Ga ELISA Kit for Alpha-1-Antitrypsin (a1AT) Enzyme-linked immunosorbent assay for Antigen Detection.
  1. "Cloning and expression in Escherichia coli of full-length complementary DNA coding for human alpha 1-antitrypsin."DNA 2:255-264(1983) [PubMed] [Europe PMC] [Abstract]
  2. "Complete sequence of the cDNA for human alpha 1-antitrypsin and the gene for the S variant."Biochemistry 23:4828-4837(1984) [PubMed] [Europe PMC] [Abstract]
  3. "Synthesis in yeast of a functional oxidation-resistant mutant of human alpha-antitrypsin."Nature 312:77-80(1984) [PubMed] [Europe PMC] [Abstract]
  4. "Cell-specific expression of a transfected human alpha 1-antitrypsin gene."Cell 41:531-540(1985) [PubMed] [Europe PMC] [Abstract]
  5. "Identification of a second mutation in the protein-coding sequence of the Z type alpha 1-antitrypsin gene."J. Biol. Chem. 261:15989-15994(1986) [PubMed] [Europe PMC] [Abstract]
  6. ErratumJ. Biol. Chem. 262:10412-10412(1987)
  7. "An alpha-1 antitrypsin genetic variant from India."Indian J. Biochem. Biophys. 44:176-178(2007) [PubMed] [Europe PMC] [Abstract]
  8. "Complete sequencing and characterization of 21,243 full-length human cDNAs." Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
  9. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
  10. "Sequence homology and structural comparison between the chromosomal human alpha 1-antitrypsin and chicken ovalbumin genes."Nature 297:655-659(1982) [PubMed] [Europe PMC] [Abstract]
  11. "The covalent structure of human alpha1-protease inhibitor."Fed. Proc. 41:1016-1016(1982)
  12. "Structure and variation of human alpha 1-antitrypsin."Nature 298:329-334(1982) [PubMed] [Europe PMC] [Abstract]
  13. "Appearance of an aberrant form of alpha-antitrypsin in the circulation of chronic cigarette smokers and its effect on the insulin induced NO synthesis in blood platelets."Submitted (AUG-2007) to UniProtKB
  14. "Characterization of a 54 kDa, alpha 1-antitrypsin-like protein isolated from ascitic fluid of an endometrial cancer patient."Jpn. J. Cancer Res. 82:693-700(1991) [PubMed] [Europe PMC] [Abstract]
  15. "Comprehensive glyco-proteomic analysis of human alpha1-antitrypsin and its charge isoforms."Proteomics 6:3369-3380(2006) [PubMed] [Europe PMC] [Abstract]
  16. "Sequencing of a urinary stone protein, identical to alpha-one antitrypsin, which lacks 22 amino acids."Biochem. Biophys. Res. Commun. 193:1049-1053(1993) [PubMed] [Europe PMC] [Abstract]
  17. , Submitted (MAR-2007) to UniProtKB
  18. "Alpha 1-antitrypsin and serum albumin mRNA accumulation in normal, acute phase and ZZ human liver."FEBS Lett. 189:361-366(1985) [PubMed] [Europe PMC] [Abstract]
  19. "Cloning and sequence of cDNA coding for alpha 1-antitrypsin."Proc. Natl. Acad. Sci. U.S.A. 78:6826-6830(1981) [PubMed] [Europe PMC] [Abstract]
  20. "Isolation and serine protease inhibitory activity of the 44-residue, C-terminal fragment of alpha 1-antitrypsin from human placenta."Matrix 12:233-241(1992) [PubMed] [Europe PMC] [Abstract]
  21. "Construction and partial characterization of a human liver cDNA library."Biomed. Biochim. Acta 44:421-431(1985) [PubMed] [Europe PMC] [Abstract]
  22. "Identification and quantification of N-linked glycoproteins using hydrazide chemistry, stable isotope labeling and mass spectrometry."Nat. Biotechnol. 21:660-666(2003) [PubMed] [Europe PMC] [Abstract]
  23. "A proteomic analysis of human bile."Mol. Cell. Proteomics 3:715-728(2004) [PubMed] [Europe PMC] [Abstract]
  24. "Screening for N-glycosylated proteins by liquid chromatography mass spectrometry."Proteomics 4:454-465(2004) [PubMed] [Europe PMC] [Abstract]
  25. "Human plasma N-glycoproteome analysis by immunoaffinity subtraction, hydrazide chemistry, and mass spectrometry."J. Proteome Res. 4:2070-2080(2005) [PubMed] [Europe PMC] [Abstract]
  26. "Elucidation of N-glycosylation sites on human platelet proteins: a glycoproteomic approach."Mol. Cell. Proteomics 5:226-233(2006) [PubMed] [Europe PMC] [Abstract]
  27. "Glycoproteomics analysis of human liver tissue by combination of multiple enzyme digestion and hydrazide chemistry."J. Proteome Res. 8:651-661(2009) [PubMed] [Europe PMC] [Abstract]
  28. "A strategy for precise and large scale identification of core fucosylated glycoproteins."Mol. Cell. Proteomics 8:913-923(2009) [PubMed] [Europe PMC] [Abstract]
  29. "Enrichment of glycopeptides for glycan structure and attachment site identification."Nat. Methods 6:809-811(2009) [PubMed] [Europe PMC] [Abstract]
  30. "Initial characterization of the human central proteome."BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]
  31. "Human urinary glycoproteomics; attachment site specific analysis of N-and O-linked glycosylations by CID and ECD."Mol. Cell. Proteomics 0:0-0(2011) [PubMed] [Europe PMC] [Abstract]
  32. "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]
  33. "Human alpha 1-proteinase inhibitor. Crystal structure analysis of two crystal modifications, molecular model and preliminary analysis of the implications for function."J. Mol. Biol. 177:531-556(1984) [PubMed] [Europe PMC] [Abstract]
  34. "The S variant of human alpha 1-antitrypsin, structure and implications for function and metabolism."Protein Eng. 2:407-415(1989) [PubMed] [Europe PMC] [Abstract]
  35. "Crystal structure of an uncleaved alpha 1-antitrypsin reveals the conformation of its inhibitory reactive loop."FEBS Lett. 377:150-154(1995) [PubMed] [Europe PMC] [Abstract]
  36. "Inhibitory conformation of the reactive loop of alpha 1-antitrypsin."Nat. Struct. Biol. 3:676-681(1996) [PubMed] [Europe PMC] [Abstract]
  37. "The native strains in the hydrophobic core and flexible reactive loop of a serine protease inhibitor: crystal structure of an uncleaved alpha1-antitrypsin at 2.7 A."Structure 4:1181-1192(1996) [PubMed] [Europe PMC] [Abstract]
  38. "Wild-type alpha 1-antitrypsin is in the canonical inhibitory conformation."J. Mol. Biol. 275:419-425(1998) [PubMed] [Europe PMC] [Abstract]
  39. "Structure of a serpin-protease complex shows inhibition by deformation."Nature 407:923-926(2000) [PubMed] [Europe PMC] [Abstract]
  40. "Cleaved antitrypsin polymers at atomic resolution."Protein Sci. 9:417-420(2000) [PubMed] [Europe PMC] [Abstract]
  41. "Topography of a 2.0 A structure of alpha1-antitrypsin reveals targets for rational drug design to prevent conformational disease."Protein Sci. 9:1274-1281(2000) [PubMed] [Europe PMC] [Abstract]
  42. "A 2.1 A resolution structure of an uncleaved alpha(1)-antitrypsin shows variability of the reactive center and other loops."J. Mol. Biol. 306:109-119(2001) [PubMed] [Europe PMC] [Abstract]
  43. "Interactions causing the kinetic trap in serpin protein folding."J. Biol. Chem. 277:46347-46354(2002) [PubMed] [Europe PMC] [Abstract]
  44. "Canonical inhibitor-like interactions explain reactivity of alpha1-proteinase inhibitor Pittsburgh and antithrombin with proteinases."J. Biol. Chem. 278:37881-37887(2003) [PubMed] [Europe PMC] [Abstract]
  45. "Alpha 1-antitrypsin: structure, function and molecular biology of the gene."Biosci. Rep. 9:129-138(1989) [PubMed] [Europe PMC] [Abstract]
  46. "The molecular genetics of alpha 1 antitrypsin deficiency."Bioessays 13:163-169(1991) [PubMed] [Europe PMC] [Abstract]
  47. "Characterization of the gene and protein of the common alpha 1-antitrypsin normal M2 allele."Am. J. Hum. Genet. 43:322-330(1988) [PubMed] [Europe PMC] [Abstract]
  48. "Characterisation of the alpha-1-antitrypsin M3 gene, a normal variant."Hum. Genet. 85:381-382(1990) [PubMed] [Europe PMC] [Abstract]
  49. "Characterization of the molecular basis of the alpha 1-antitrypsin F allele."Am. J. Hum. Genet. 48:1154-1158(1991) [PubMed] [Europe PMC] [Abstract]
  50. "A Pro-->Leu substitution in codon 369 of the alpha-1-antitrypsin deficiency variant PI M-Heerlen."Hum. Genet. 81:264-268(1989) [PubMed] [Europe PMC] [Abstract]
  51. "In-frame single codon deletion in the M-Malton deficiency allele of alpha 1-antitrypsin."Am. J. Hum. Genet. 44:894-902(1989) [PubMed] [Europe PMC] [Abstract]
  52. "Molecular basis of alpha 1-antitrypsin deficiency and emphysema associated with the alpha 1-antitrypsin M-Mineral springs allele."Mol. Cell. Biol. 10:47-56(1990) [PubMed] [Europe PMC] [Abstract]
  53. "Molecular analysis of the gene of the alpha 1-antitrypsin deficiency variant, M-Nichinan."Am. J. Hum. Genet. 46:602-612(1990) [PubMed] [Europe PMC] [Abstract]
  54. "Characterization of the gene and protein of the alpha 1-antitrypsin 'deficiency' allele M-Procida."J. Biol. Chem. 263:15528-15534(1988) [PubMed] [Europe PMC] [Abstract]
  55. "Genetic diversity from a limited repertoire of mutations on different common allelic backgrounds: alpha 1-antitrypsin deficiency variant P-Duarte."Hum. Mutat. 2:221-228(1993) [PubMed] [Europe PMC] [Abstract]
  56. "Mutation of antitrypsin to antithrombin. Alpha 1-antitrypsin Pittsburgh (358 Met leads to Arg), a fatal bleeding disorder."N. Engl. J. Med. 309:694-698(1983) [PubMed] [Europe PMC] [Abstract]
  57. "Siiyama (serine 53 (TCC) to phenylalanine 53 (TTC)). A new alpha 1-antitrypsin-deficient variant with mutation on a predicted conserved residue of the serpin backbone."J. Biol. Chem. 266:12627-12632(1991) [PubMed] [Europe PMC] [Abstract]
  58. "Characterization of the normal alpha 1-antitrypsin allele V-Munich: a variant associated with a unique protein isoelectric focusing pattern."Am. J. Hum. Genet. 46:810-816(1990) [PubMed] [Europe PMC] [Abstract]
  59. "Alpha 1-antitrypsin W-Bethesda: molecular basis of an unusual alpha 1-antitrypsin deficiency variant."Biochem. Biophys. Res. Commun. 170:1013-1020(1990) [PubMed] [Europe PMC] [Abstract]
  60. "Sequence data of the rare deficient alpha 1-antitrypsin variant PI Zaugsburg."Am. J. Hum. Genet. 46:1158-1162(1990) [PubMed] [Europe PMC] [Abstract]
  61. "Molecular characterisation of two alpha-1-antitrypsin deficiency variants: proteinase inhibitor (Pi) Null(Newport) (Gly115-->Ser) and (Pi) Z Wrexham (Ser-19-->Leu)."Hum. Genet. 85:537-540(1990) [PubMed] [Europe PMC] [Abstract]
  62. "Molecular characterisation of three alpha-1-antitrypsin deficiency variants: proteinase inhibitor (Pi) nullcardiff (Asp256-->Val); PiM-Malton (Phe51-->deletion) and PiI (Arg39-->Cys)."Hum. Genet. 84:55-58(1989) [PubMed] [Europe PMC] [Abstract]
  63. "A null deficiency allele of alpha 1-antitrypsin, QO-Ludwigshafen, with altered tertiary structure."J. Clin. Invest. 86:1878-1884(1990) [PubMed] [Europe PMC] [Abstract]
  64. "Identification and DNA sequence analysis of 15 new alpha 1-antitrypsin variants, including two PI*Q0 alleles and one deficient PI*M allele."Am. J. Hum. Genet. 55:1113-1121(1994) [PubMed] [Europe PMC] [Abstract]
  65. "A new alpha 1-antitrypsin mutation, Thr-Met 85, (PI ZBristol) associated with novel electrophoretic properties."Ann. Hum. Genet. 61:385-391(1997) [PubMed] [Europe PMC] [Abstract]
  66. "Identification and molecular characterization of the new alpha-1-antitrypsin deficient allele PI YBarcelona (Asp256Val and Pro391His)."Hum. Mutat. 12:213-213(1998) [PubMed] [Europe PMC] [Abstract]
  67. "A novel alpha-1-antitrypsin P362H variant found in a population sample from Sao Tome e Principe (Gulf of Guinea, West Africa)."Hum. Mutat. 13:414-414(1999)
  68. "A novel alpha-1-antitrypsin r281del variant found in a population sample from the Basque country."Hum. Mutat. 15:121-122(2000) [PubMed] [Europe PMC] [Abstract]
  69. "SERPINA2 is a novel gene with a divergent function from SERPINA1."PLoS ONE 8:E66889-E66889(2013) [PubMed] [Europe PMC] [Abstract]