Details for: SERPINA1

Gene ID: 5265

Symbol: SERPINA1

Ensembl ID: ENSG00000197249

Description: serpin family A member 1

Associated with

Cells (max top 100)

(Cell Significance Index and respective Thresholds are uniquely calculated using our advanced thresholding algorithms to reveal cell-specific gene markers)

  • Cell Name: polychromatophilic erythroblast (CL0000550)
    Fold Change: 181.1049
    Cell Significance Index: -28.1700
  • Cell Name: hematopoietic oligopotent progenitor cell (CL0002032)
    Fold Change: 113.4270
    Cell Significance Index: -28.7700
  • Cell Name: mucosal type mast cell (CL0000485)
    Fold Change: 82.1032
    Cell Significance Index: -33.3600
  • Cell Name: peripheral blood mononuclear cell (CL2000001)
    Fold Change: 71.7361
    Cell Significance Index: -36.9000
  • Cell Name: obsolete epithelial cell of alveolus of lung (CL0010003)
    Fold Change: 55.3649
    Cell Significance Index: 1380.6400
  • Cell Name: ileal goblet cell (CL1000326)
    Fold Change: 48.7614
    Cell Significance Index: -32.7200
  • Cell Name: periportal region hepatocyte (CL0019026)
    Fold Change: 48.1511
    Cell Significance Index: 710.7100
  • Cell Name: ciliated cell of the bronchus (CL0002332)
    Fold Change: 34.7214
    Cell Significance Index: -33.1500
  • Cell Name: orthochromatic erythroblast (CL0000552)
    Fold Change: 22.7664
    Cell Significance Index: -28.0700
  • Cell Name: centrilobular region hepatocyte (CL0019029)
    Fold Change: 15.3586
    Cell Significance Index: 258.7200
  • Cell Name: CD8-alpha-beta-positive, alpha-beta intraepithelial T cell (CL0000796)
    Fold Change: 12.4867
    Cell Significance Index: -33.4500
  • Cell Name: CD14-positive, CD16-negative classical monocyte (CL0002057)
    Fold Change: 12.2098
    Cell Significance Index: 225.6700
  • Cell Name: early T lineage precursor (CL0002425)
    Fold Change: 10.5623
    Cell Significance Index: 153.3500
  • Cell Name: stromal cell of bone marrow (CL0010001)
    Fold Change: 7.2858
    Cell Significance Index: -28.7500
  • Cell Name: neoplastic cell (CL0001063)
    Fold Change: 6.4974
    Cell Significance Index: 1289.4300
  • Cell Name: hepatic pit cell (CL2000054)
    Fold Change: 5.5676
    Cell Significance Index: 14.9200
  • Cell Name: midzonal region hepatocyte (CL0019028)
    Fold Change: 5.0454
    Cell Significance Index: 29.0600
  • Cell Name: microfold cell of epithelium of small intestine (CL1000353)
    Fold Change: 3.4874
    Cell Significance Index: 241.1700
  • Cell Name: colon goblet cell (CL0009039)
    Fold Change: 2.8266
    Cell Significance Index: 279.6100
  • Cell Name: pulmonary alveolar epithelial cell (CL0000322)
    Fold Change: 2.7717
    Cell Significance Index: 2097.9700
  • Cell Name: endothelial cell of periportal hepatic sinusoid (CL0019021)
    Fold Change: 2.7666
    Cell Significance Index: 9.6300
  • Cell Name: intrahepatic cholangiocyte (CL0002538)
    Fold Change: 2.3817
    Cell Significance Index: 8.9700
  • Cell Name: endothelial cell of pericentral hepatic sinusoid (CL0019022)
    Fold Change: 1.8785
    Cell Significance Index: 14.8300
  • Cell Name: luminal hormone-sensing cell of mammary gland (CL4033058)
    Fold Change: 1.7573
    Cell Significance Index: 10.8200
  • Cell Name: tuft cell of small intestine (CL0009080)
    Fold Change: 1.6242
    Cell Significance Index: 16.3900
  • Cell Name: intestinal crypt stem cell of colon (CL0009043)
    Fold Change: 1.5403
    Cell Significance Index: 167.5400
  • Cell Name: epithelial cell of small intestine (CL0002254)
    Fold Change: 1.4840
    Cell Significance Index: 241.3700
  • Cell Name: tuft cell of colon (CL0009041)
    Fold Change: 1.4241
    Cell Significance Index: 1285.8500
  • Cell Name: gut absorptive cell (CL0000677)
    Fold Change: 1.3344
    Cell Significance Index: 80.1100
  • Cell Name: progenitor cell of mammary luminal epithelium (CL0009116)
    Fold Change: 1.2706
    Cell Significance Index: 94.7000
  • Cell Name: cell in vitro (CL0001034)
    Fold Change: 1.2255
    Cell Significance Index: 669.2800
  • Cell Name: pancreatic acinar cell (CL0002064)
    Fold Change: 0.1222
    Cell Significance Index: 20.8600
  • Cell Name: neutrophil progenitor cell (CL0000834)
    Fold Change: 0.1202
    Cell Significance Index: 3.2200
  • Cell Name: enterocyte of epithelium of small intestine (CL1000334)
    Fold Change: 0.1076
    Cell Significance Index: 3.1000
  • Cell Name: intestinal epithelial cell (CL0002563)
    Fold Change: 0.0971
    Cell Significance Index: 1.0100
  • Cell Name: abnormal cell (CL0001061)
    Fold Change: 0.0903
    Cell Significance Index: 9.2200
  • Cell Name: enteroendocrine cell of colon (CL0009042)
    Fold Change: 0.0891
    Cell Significance Index: 16.9500
  • Cell Name: fallopian tube secretory epithelial cell (CL4030006)
    Fold Change: 0.0672
    Cell Significance Index: 1.0400
  • Cell Name: cholangiocyte (CL1000488)
    Fold Change: 0.0527
    Cell Significance Index: 0.5200
  • Cell Name: enterocyte of epithelium of large intestine (CL0002071)
    Fold Change: 0.0154
    Cell Significance Index: 0.7000
  • Cell Name: pancreatic PP cell (CL0002275)
    Fold Change: 0.0149
    Cell Significance Index: 9.3100
  • Cell Name: neuron associated cell (CL0000095)
    Fold Change: -0.0016
    Cell Significance Index: -0.0600
  • Cell Name: pancreatic A cell (CL0000171)
    Fold Change: -0.0024
    Cell Significance Index: -1.8100
  • Cell Name: pancreatic D cell (CL0000173)
    Fold Change: -0.0138
    Cell Significance Index: -2.9000
  • Cell Name: pigmented epithelial cell (CL0000529)
    Fold Change: -0.0151
    Cell Significance Index: -28.4500
  • Cell Name: anterior lens cell (CL0002223)
    Fold Change: -0.0228
    Cell Significance Index: -42.1200
  • Cell Name: pancreatic ductal cell (CL0002079)
    Fold Change: -0.0235
    Cell Significance Index: -2.6900
  • Cell Name: lens epithelial cell (CL0002224)
    Fold Change: -0.0275
    Cell Significance Index: -42.3400
  • Cell Name: type B pancreatic cell (CL0000169)
    Fold Change: -0.0329
    Cell Significance Index: -18.5800
  • Cell Name: intestinal crypt stem cell of small intestine (CL0009017)
    Fold Change: -0.0409
    Cell Significance Index: -0.8700
  • Cell Name: kidney loop of Henle cortical thick ascending limb epithelial cell (CL1001109)
    Fold Change: -0.0417
    Cell Significance Index: -30.5400
  • Cell Name: non-pigmented ciliary epithelial cell (CL0002304)
    Fold Change: -0.0547
    Cell Significance Index: -34.7300
  • Cell Name: hair follicular keratinocyte (CL2000092)
    Fold Change: -0.0667
    Cell Significance Index: -29.5100
  • Cell Name: endothelial cell of hepatic sinusoid (CL1000398)
    Fold Change: -0.0721
    Cell Significance Index: -0.6900
  • Cell Name: dopaminergic neuron (CL0000700)
    Fold Change: -0.0803
    Cell Significance Index: -23.1000
  • Cell Name: ciliary muscle cell (CL1000443)
    Fold Change: -0.0846
    Cell Significance Index: -38.4000
  • Cell Name: obsolete caudal ganglionic eminence derived GABAergic cortical interneuron (CL4023070)
    Fold Change: -0.0873
    Cell Significance Index: -31.3100
  • Cell Name: enteroendocrine cell of small intestine (CL0009006)
    Fold Change: -0.1088
    Cell Significance Index: -2.7200
  • Cell Name: lactocyte (CL0002325)
    Fold Change: -0.1105
    Cell Significance Index: -14.2700
  • Cell Name: pancreatic endocrine cell (CL0008024)
    Fold Change: -0.1147
    Cell Significance Index: -13.0900
  • Cell Name: intermediate cell of urothelium (CL4030055)
    Fold Change: -0.1315
    Cell Significance Index: -23.7000
  • Cell Name: luminal adaptive secretory precursor cell of mammary gland (CL4033057)
    Fold Change: -0.1343
    Cell Significance Index: -6.3100
  • Cell Name: L2/3-6 intratelencephalic projecting glutamatergic neuron (CL4023040)
    Fold Change: -0.1537
    Cell Significance Index: -30.8400
  • Cell Name: monocyte-derived dendritic cell (CL0011031)
    Fold Change: -0.1537
    Cell Significance Index: -2.6400
  • Cell Name: epithelial cell of stomach (CL0002178)
    Fold Change: -0.1775
    Cell Significance Index: -20.6800
  • Cell Name: stromal cell of ovary (CL0002132)
    Fold Change: -0.1890
    Cell Significance Index: -25.9600
  • Cell Name: transit amplifying cell of colon (CL0009011)
    Fold Change: -0.2004
    Cell Significance Index: -6.4200
  • Cell Name: conjunctival epithelial cell (CL1000432)
    Fold Change: -0.2184
    Cell Significance Index: -2.9800
  • Cell Name: basal cell of urothelium (CL1000486)
    Fold Change: -0.2208
    Cell Significance Index: -27.1500
  • Cell Name: basal epithelial cell of tracheobronchial tree (CL0002329)
    Fold Change: -0.2295
    Cell Significance Index: -6.4200
  • Cell Name: pigmented ciliary epithelial cell (CL0002303)
    Fold Change: -0.2365
    Cell Significance Index: -34.3800
  • Cell Name: hepatoblast (CL0005026)
    Fold Change: -0.2420
    Cell Significance Index: -4.0700
  • Cell Name: BEST4+ enteroycte (CL4030026)
    Fold Change: -0.2475
    Cell Significance Index: -3.7300
  • Cell Name: skeletal muscle fiber (CL0008002)
    Fold Change: -0.2568
    Cell Significance Index: -6.6000
  • Cell Name: smooth muscle cell of sphincter of pupil (CL0002243)
    Fold Change: -0.2763
    Cell Significance Index: -28.7700
  • Cell Name: tonsil germinal center B cell (CL2000006)
    Fold Change: -0.2764
    Cell Significance Index: -32.6000
  • Cell Name: eukaryotic cell (CL0000255)
    Fold Change: -0.2992
    Cell Significance Index: -13.0100
  • Cell Name: lung endothelial cell (CL1001567)
    Fold Change: -0.3118
    Cell Significance Index: -16.2400
  • Cell Name: CD14-positive monocyte (CL0001054)
    Fold Change: -0.3140
    Cell Significance Index: -6.1400
  • Cell Name: kidney loop of Henle descending limb epithelial cell (CL1001021)
    Fold Change: -0.3643
    Cell Significance Index: -28.8600
  • Cell Name: hepatocyte (CL0000182)
    Fold Change: -0.3699
    Cell Significance Index: -5.1300
  • Cell Name: cardiac muscle myoblast (CL0000513)
    Fold Change: -0.4911
    Cell Significance Index: -37.6900
  • Cell Name: odontoblast (CL0000060)
    Fold Change: -0.4996
    Cell Significance Index: -64.0500
  • Cell Name: intestinal tuft cell (CL0019032)
    Fold Change: -0.5335
    Cell Significance Index: -32.7100
  • Cell Name: early pro-B cell (CL0002046)
    Fold Change: -0.5762
    Cell Significance Index: -37.1800
  • Cell Name: pancreatic epsilon cell (CL0005019)
    Fold Change: -0.5763
    Cell Significance Index: -5.3400
  • Cell Name: eye photoreceptor cell (CL0000287)
    Fold Change: -0.5765
    Cell Significance Index: -36.3400
  • Cell Name: retinal progenitor cell (CL0002672)
    Fold Change: -0.5843
    Cell Significance Index: -32.7900
  • Cell Name: bladder urothelial cell (CL1001428)
    Fold Change: -0.5970
    Cell Significance Index: -31.0100
  • Cell Name: paneth cell of colon (CL0009009)
    Fold Change: -0.6026
    Cell Significance Index: -9.0300
  • Cell Name: proerythroblast (CL0000547)
    Fold Change: -0.6109
    Cell Significance Index: -8.7600
  • Cell Name: glycinergic neuron (CL1001509)
    Fold Change: -0.6217
    Cell Significance Index: -32.6400
  • Cell Name: endothelial cell of venule (CL1000414)
    Fold Change: -0.6391
    Cell Significance Index: -7.2600
  • Cell Name: classical monocyte (CL0000860)
    Fold Change: -0.7368
    Cell Significance Index: -6.8600
  • Cell Name: CD14-low, CD16-positive monocyte (CL0002396)
    Fold Change: -0.7415
    Cell Significance Index: -17.9600
  • Cell Name: indirect pathway medium spiny neuron (CL4023029)
    Fold Change: -0.7505
    Cell Significance Index: -33.2000
  • Cell Name: small intestine goblet cell (CL1000495)
    Fold Change: -0.7681
    Cell Significance Index: -26.9900
  • Cell Name: Kupffer cell (CL0000091)
    Fold Change: -0.7917
    Cell Significance Index: -7.2500
  • Cell Name: cortical cell of adrenal gland (CL0002097)
    Fold Change: -0.8166
    Cell Significance Index: -21.8800
  • Cell Name: goblet cell (CL0000160)
    Fold Change: -0.8281
    Cell Significance Index: -7.4900

Cell ID: Standard Cell Ontology term used for mapping and comparing cells across experiments. Ensures consistency in analyzing cellular functions across tissues.
Fold Change: Represents the ratio of the current Cell Significance Index to the Cell Significance Index Threshold, indicating how much the gene expression has changed compared to a baseline.
Cell Significance Index: Reflects how strongly a gene is expressed in this specific cell.

Cell ID: Standard Cell Ontology term used for mapping and comparing cells across experiments. Ensures consistency in analyzing cellular functions across tissues.
Fold Change: Represents the ratio of the current Cell Significance Index to the Cell Significance Index Threshold, indicating how much the gene expression has changed compared to a baseline.
Cell Significance Index: Reflects how strongly a gene is expressed in this cell type. Calculated using techniques like effect size estimation and bootstrapping for reliability.

Cell ID: Standard Cell Ontology term used for mapping and comparing cells across experiments. Ensures consistency in analyzing cellular functions across tissues.
Fold Change: Represents the ratio of the current Cell Significance Index to the Cell Significance Index Threshold, indicating how much the gene expression has changed compared to a baseline.
Cell Significance Index: Reflects how strongly a gene is expressed in this cell type. Calculated using techniques like effect size estimation and bootstrapping for reliability.

Other Information

**Key Characteristics** 1. **Protease Inhibitor Function**: SERPINA1 is a serine protease inhibitor, which means it binds to and inhibits the activity of proteolytic enzymes, thereby protecting tissues from damage. 2. **Alpha-1 Antitrypsin**: SERPINA1 is also known as Alpha-1 Antitrypsin, a designation that reflects its primary function in protecting the lungs from the enzyme neutrophil elastase. 3. **Glycosylation**: SERPINA1 is a glycoprotein, with a complex glycosylation pattern that affects its stability, function, and interaction with other proteins. 4. **Cellular Expression**: SERPINA1 is expressed in various cell types, including hepatocytes, monocytes, pneumocytes, and endothelial cells, suggesting a broad range of physiological and pathological roles. **Pathways and Functions** 1. **Acute-Phase Response**: SERPINA1 is induced during the acute-phase response, a systemic reaction to inflammation, injury, or infection. This induction is mediated by cytokines and other signaling molecules. 2. **Blood Coagulation**: SERPINA1 has been implicated in the regulation of blood coagulation, with evidence suggesting that it inhibits the activity of coagulation factors and platelet activators. 3. **Endoplasmic Reticulum (ER) Quality Control**: SERPINA1 is synthesized in the ER and plays a role in maintaining ER homeostasis, particularly in the context of glycosylation and protein folding. 4. **Immune System**: SERPINA1 has been shown to modulate immune responses, including the regulation of neutrophil degranulation and platelet activation. 5. **Protease Regulation**: SERPINA1 inhibits the activity of various proteases, including serine proteases, aspartic proteases, and cysteine proteases, thereby protecting tissues from proteolytic damage. **Clinical Significance** 1. **Alpha-1 Antitrypsin Deficiency**: Mutations in the SERPINA1 gene can lead to Alpha-1 Antitrypsin Deficiency (A1AD), a genetic disorder characterized by chronic lung disease, liver disease, and other extrapulmonary manifestations. 2. **Lung Disease**: SERPINA1 plays a critical role in protecting the lungs from damage caused by proteolytic enzymes, and its deficiency is associated with chronic obstructive pulmonary disease (COPD) and emphysema. 3. **Liver Disease**: A1AD can also lead to liver disease, including cirrhosis and liver failure, due to the accumulation of mutant SERPINA1 protein in hepatocytes. 4. **Other Diseases**: SERPINA1 has been implicated in various other diseases, including cancer, cardiovascular disease, and neurodegenerative disorders, highlighting its potential role as a biomarker or therapeutic target. In conclusion, SERPINA1 is a multifunctional protein that plays a critical role in protecting tissues from proteolytic damage. Its dysregulation can lead to various diseases, including Alpha-1 Antitrypsin Deficiency and other extrapulmonary manifestations. Further research is needed to fully elucidate the functions and clinical significance of SERPINA1.

Genular Protein ID: 216985202

Symbol: A1AT_HUMAN

Name: Alpha-1 protease inhibitor

UniProtKB Accession Codes:

Database IDs:

Citations:

PubMed ID: 6319097

Title: Cloning and expression in Escherichia coli of full-length complementary DNA coding for human alpha 1-antitrypsin.

PubMed ID: 6319097

DOI: 10.1089/dna.1983.2.255

PubMed ID: 6093867

Title: Complete sequence of the cDNA for human alpha 1-antitrypsin and the gene for the S variant.

PubMed ID: 6093867

DOI: 10.1021/bi00316a003

PubMed ID: 6387509

Title: Synthesis in yeast of a functional oxidation-resistant mutant of human alpha-antitrypsin.

PubMed ID: 6387509

DOI: 10.1038/312077a0

PubMed ID: 2985281

Title: Cell-specific expression of a transfected human alpha 1-antitrypsin gene.

PubMed ID: 2985281

DOI: 10.1016/s0092-8674(85)80026-x

PubMed ID: 3491072

Title: Identification of a second mutation in the protein-coding sequence of the Z type alpha 1-antitrypsin gene.

PubMed ID: 3491072

DOI: 10.1016/s0021-9258(18)66664-5

PubMed ID: 17650587

Title: An alpha-1 antitrypsin genetic variant from India.

PubMed ID: 17650587

PubMed ID: 14702039

Title: Complete sequencing and characterization of 21,243 full-length human cDNAs.

PubMed ID: 14702039

DOI: 10.1038/ng1285

PubMed ID: 15489334

Title: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).

PubMed ID: 15489334

DOI: 10.1101/gr.2596504

PubMed ID: 6979715

Title: Sequence homology and structural comparison between the chromosomal human alpha 1-antitrypsin and chicken ovalbumin genes.

PubMed ID: 6979715

DOI: 10.1038/297655a0

PubMed ID: 7045697

Title: Structure and variation of human alpha 1-antitrypsin.

PubMed ID: 7045697

DOI: 10.1038/298329a0

PubMed ID: 1906855

Title: Characterization of a 54 kDa, alpha 1-antitrypsin-like protein isolated from ascitic fluid of an endometrial cancer patient.

PubMed ID: 1906855

DOI: 10.1111/j.1349-7006.1991.tb01905.x

PubMed ID: 16622833

Title: Comprehensive glyco-proteomic analysis of human alpha1-antitrypsin and its charge isoforms.

PubMed ID: 16622833

DOI: 10.1002/pmic.200500751

PubMed ID: 8323530

Title: Sequencing of a urinary stone protein, identical to alpha-one antitrypsin, which lacks 22 amino acids.

PubMed ID: 8323530

DOI: 10.1006/bbrc.1993.1731

PubMed ID: 3876243

Title: Alpha 1-antitrypsin and serum albumin mRNA accumulation in normal, acute phase and ZZ human liver.

PubMed ID: 3876243

DOI: 10.1016/0014-5793(85)81056-5

PubMed ID: 7031661

Title: Cloning and sequence of cDNA coding for alpha 1-antitrypsin.

PubMed ID: 7031661

DOI: 10.1073/pnas.78.11.6826

PubMed ID: 1406456

Title: Isolation and serine protease inhibitory activity of the 44-residue, C-terminal fragment of alpha 1-antitrypsin from human placenta.

PubMed ID: 1406456

DOI: 10.1016/s0934-8832(11)80066-1

PubMed ID: 3873938

Title: Construction and partial characterization of a human liver cDNA library.

PubMed ID: 3873938

PubMed ID: 3533918

Title: The inactivation of human plasma alpha 1-proteinase inhibitor by proteinases from Staphylococcus aureus.

PubMed ID: 3533918

DOI: 10.1016/s0021-9258(18)67022-x

PubMed ID: 12754519

Title: Identification and quantification of N-linked glycoproteins using hydrazide chemistry, stable isotope labeling and mass spectrometry.

PubMed ID: 12754519

DOI: 10.1038/nbt827

PubMed ID: 15084671

Title: A proteomic analysis of human bile.

PubMed ID: 15084671

DOI: 10.1074/mcp.m400015-mcp200

PubMed ID: 14760718

Title: Screening for N-glycosylated proteins by liquid chromatography mass spectrometry.

PubMed ID: 14760718

DOI: 10.1002/pmic.200300556

PubMed ID: 16335952

Title: Human plasma N-glycoproteome analysis by immunoaffinity subtraction, hydrazide chemistry, and mass spectrometry.

PubMed ID: 16335952

DOI: 10.1021/pr0502065

PubMed ID: 16263699

Title: Elucidation of N-glycosylation sites on human platelet proteins: a glycoproteomic approach.

PubMed ID: 16263699

DOI: 10.1074/mcp.m500324-mcp200

PubMed ID: 19159218

Title: Glycoproteomics analysis of human liver tissue by combination of multiple enzyme digestion and hydrazide chemistry.

PubMed ID: 19159218

DOI: 10.1021/pr8008012

PubMed ID: 19139490

Title: A strategy for precise and large scale identification of core fucosylated glycoproteins.

PubMed ID: 19139490

DOI: 10.1074/mcp.m800504-mcp200

PubMed ID: 19838169

Title: Enrichment of glycopeptides for glycan structure and attachment site identification.

PubMed ID: 19838169

DOI: 10.1038/nmeth.1392

PubMed ID: 21269460

Title: Initial characterization of the human central proteome.

PubMed ID: 21269460

DOI: 10.1186/1752-0509-5-17

PubMed ID: 22171320

Title: Human urinary glycoproteomics; attachment site specific analysis of N- and O-linked glycosylations by CID and ECD.

PubMed ID: 22171320

DOI: 10.1074/mcp.m111.013649

PubMed ID: 24275569

Title: An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome.

PubMed ID: 24275569

DOI: 10.1016/j.jprot.2013.11.014

PubMed ID: 26091039

Title: A single kinase generates the majority of the secreted phosphoproteome.

PubMed ID: 26091039

DOI: 10.1016/j.cell.2015.05.028

PubMed ID: 25944712

Title: N-terminome analysis of the human mitochondrial proteome.

PubMed ID: 25944712

DOI: 10.1002/pmic.201400617

PubMed ID: 31358993

Title: CELA2A mutations predispose to early-onset atherosclerosis and metabolic syndrome and affect plasma insulin and platelet activation.

PubMed ID: 31358993

DOI: 10.1038/s41588-019-0470-3

PubMed ID: 31142615

Title: The E3 ubiquitin ligase MARCH2 regulates ERGIC3-dependent trafficking of secretory proteins.

PubMed ID: 31142615

DOI: 10.1074/jbc.ra119.007435

PubMed ID: 6332197

Title: Human alpha 1-proteinase inhibitor. Crystal structure analysis of two crystal modifications, molecular model and preliminary analysis of the implications for function.

PubMed ID: 6332197

DOI: 10.1016/0022-2836(84)90298-5

PubMed ID: 2785270

Title: The S variant of human alpha 1-antitrypsin, structure and implications for function and metabolism.

PubMed ID: 2785270

DOI: 10.1093/protein/2.6.407

PubMed ID: 8543039

Title: Crystal structure of an uncleaved alpha 1-antitrypsin reveals the conformation of its inhibitory reactive loop.

PubMed ID: 8543039

DOI: 10.1016/0014-5793(95)01331-8

PubMed ID: 8756325

Title: Inhibitory conformation of the reactive loop of alpha 1-antitrypsin.

PubMed ID: 8756325

DOI: 10.1038/nsb0896-676

PubMed ID: 8939743

Title: 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.

PubMed ID: 8939743

DOI: 10.1016/s0969-2126(96)00126-8

PubMed ID: 9466920

Title: Wild-type alpha 1-antitrypsin is in the canonical inhibitory conformation.

PubMed ID: 9466920

DOI: 10.1006/jmbi.1997.1458

PubMed ID: 11057674

Title: Structure of a serpin-protease complex shows inhibition by deformation.

PubMed ID: 11057674

DOI: 10.1038/35038119

PubMed ID: 10716194

Title: Cleaved antitrypsin polymers at atomic resolution.

PubMed ID: 10716194

DOI: 10.1110/ps.9.2.417

PubMed ID: 10933492

Title: Topography of a 2.0 A structure of alpha1-antitrypsin reveals targets for rational drug design to prevent conformational disease.

PubMed ID: 10933492

DOI: 10.1110/ps.9.7.1274

PubMed ID: 11178897

Title: A 2.1 A resolution structure of an uncleaved alpha(1)-antitrypsin shows variability of the reactive center and other loops.

PubMed ID: 11178897

DOI: 10.1006/jmbi.2000.4357

PubMed ID: 12244055

Title: Interactions causing the kinetic trap in serpin protein folding.

PubMed ID: 12244055

DOI: 10.1074/jbc.m207682200

PubMed ID: 12860985

Title: Canonical inhibitor-like interactions explain reactivity of alpha1-proteinase inhibitor Pittsburgh and antithrombin with proteinases.

PubMed ID: 12860985

DOI: 10.1074/jbc.m305195200

PubMed ID: 2669992

Title: Alpha 1-antitrypsin: structure, function and molecular biology of the gene.

PubMed ID: 2669992

DOI: 10.1007/bf01115992

PubMed ID: 1859394

Title: The molecular genetics of alpha 1 antitrypsin deficiency.

PubMed ID: 1859394

DOI: 10.1002/bies.950130404

PubMed ID: 2901226

Title: Characterization of the gene and protein of the common alpha 1-antitrypsin normal M2 allele.

PubMed ID: 2901226

PubMed ID: 2394452

Title: Characterisation of the alpha-1-antitrypsin M3 gene, a normal variant.

PubMed ID: 2394452

DOI: 10.1007/bf00206766

PubMed ID: 2035534

Title: Characterization of the molecular basis of the alpha 1-antitrypsin F allele.

PubMed ID: 2035534

PubMed ID: 2784123

Title: A Pro-->Leu substitution in codon 369 of the alpha-1-antitrypsin deficiency variant PI M-Heerlen.

PubMed ID: 2784123

DOI: 10.1007/bf00279001

PubMed ID: 2786335

Title: In-frame single codon deletion in the M-Malton deficiency allele of alpha 1-antitrypsin.

PubMed ID: 2786335

PubMed ID: 1967187

Title: Molecular basis of alpha 1-antitrypsin deficiency and emphysema associated with the alpha 1-antitrypsin M-Mineral springs allele.

PubMed ID: 1967187

DOI: 10.1128/mcb.10.1.47-56.1990

PubMed ID: 2309708

Title: Molecular analysis of the gene of the alpha 1-antitrypsin deficiency variant, M-Nichinan.

PubMed ID: 2309708

PubMed ID: 3262617

Title: Characterization of the gene and protein of the alpha 1-antitrypsin 'deficiency' allele M-Procida.

PubMed ID: 3262617

DOI: 10.1016/s0021-9258(19)37620-3

PubMed ID: 8364590

Title: Genetic diversity from a limited repertoire of mutations on different common allelic backgrounds: alpha 1-antitrypsin deficiency variant P-Duarte.

PubMed ID: 8364590

DOI: 10.1002/humu.1380020311

PubMed ID: 6604220

Title: Mutation of antitrypsin to antithrombin. Alpha 1-antitrypsin Pittsburgh (358 Met leads to Arg), a fatal bleeding disorder.

PubMed ID: 6604220

DOI: 10.1056/nejm198309223091203

PubMed ID: 1905728

Title: 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.

PubMed ID: 1905728

DOI: 10.1016/s0021-9258(18)98945-3

PubMed ID: 2316526

Title: Characterization of the normal alpha 1-antitrypsin allele V-Munich: a variant associated with a unique protein isoelectric focusing pattern.

PubMed ID: 2316526

PubMed ID: 2390072

Title: Alpha 1-antitrypsin W-Bethesda: molecular basis of an unusual alpha 1-antitrypsin deficiency variant.

PubMed ID: 2390072

DOI: 10.1016/0006-291x(90)90493-7

PubMed ID: 2339709

Title: Sequence data of the rare deficient alpha 1-antitrypsin variant PI Zaugsburg.

PubMed ID: 2339709

PubMed ID: 2227940

Title: Molecular characterisation of two alpha-1-antitrypsin deficiency variants: proteinase inhibitor (Pi) Null(Newport) (Gly115-->Ser) and (Pi) Z Wrexham (Ser-19-->Leu).

PubMed ID: 2227940

DOI: 10.1007/bf00194233

PubMed ID: 2606478

Title: Molecular characterisation of three alpha-1-antitrypsin deficiency variants: proteinase inhibitor (Pi) nullcardiff (Asp256-->Val); PiM-Malton (Phe51-->deletion) and PiI (Arg39-->Cys).

PubMed ID: 2606478

DOI: 10.1007/bf00210671

PubMed ID: 2254451

Title: A null deficiency allele of alpha 1-antitrypsin, QO-Ludwigshafen, with altered tertiary structure.

PubMed ID: 2254451

DOI: 10.1172/jci114919

PubMed ID: 7977369

Title: Identification and DNA sequence analysis of 15 new alpha 1-antitrypsin variants, including two PI*Q0 alleles and one deficient PI*M allele.

PubMed ID: 7977369

PubMed ID: 9459000

Title: A new alpha 1-antitrypsin mutation, Thr-Met 85, (PI ZBristol) associated with novel electrophoretic properties.

PubMed ID: 9459000

DOI: 10.1046/j.1469-1809.1997.6150385.x

PubMed ID: 10651487

Title: Identification and molecular characterization of the new alpha-1-antitrypsin deficient allele PI YBarcelona (Asp256Val and Pro391His).

PubMed ID: 10651487

PubMed ID: 10612848

Title: A novel alpha-1-antitrypsin r281del variant found in a population sample from the Basque country.

PubMed ID: 10612848

DOI: 10.1002/(sici)1098-1004(200001)15:1<121::aid-humu37>3.0.co;2-u

PubMed ID: 23826168

Title: SERPINA2 is a novel gene with a divergent function from SERPINA1.

PubMed ID: 23826168

DOI: 10.1371/journal.pone.0066889

PubMed ID: 26797521

Title: The M358R variant of alpha(1)-proteinase inhibitor inhibits coagulation factor VIIa.

PubMed ID: 26797521

DOI: 10.1016/j.bbrc.2016.01.069

Sequence Information:

  • Length: 418
  • Mass: 46737
  • Checksum: 7016555F273B7F16
  • Sequence:
  • MPSSVSWGIL LLAGLCCLVP VSLAEDPQGD AAQKTDTSHH DQDHPTFNKI TPNLAEFAFS 
    LYRQLAHQSN STNIFFSPVS IATAFAMLSL GTKADTHDEI LEGLNFNLTE IPEAQIHEGF 
    QELLRTLNQP DSQLQLTTGN GLFLSEGLKL VDKFLEDVKK LYHSEAFTVN FGDTEEAKKQ 
    INDYVEKGTQ GKIVDLVKEL DRDTVFALVN YIFFKGKWER PFEVKDTEEE DFHVDQVTTV 
    KVPMMKRLGM FNIQHCKKLS SWVLLMKYLG NATAIFFLPD EGKLQHLENE LTHDIITKFL 
    ENEDRRSASL HLPKLSITGT YDLKSVLGQL GITKVFSNGA DLSGVTEEAP LKLSKAVHKA 
    VLTIDEKGTE AAGAMFLEAI PMSIPPEVKF NKPFVFLMIE QNTKSPLFMG KVVNPTQK

Database document:

This is a preview of the gene's schema. Only a few entries are kept for 'singleCellExpressions,' 'mRNAExpressions,' and other large data arrays for visualization purposes. You can zoom in with the mouse wheel for a closer view, and the text will adjust automatically if necessary. For the full schema, download it here.