UniProtKB - P01009 (A1AT_HUMAN)
Alpha-1-antitrypsin
SERPINA1
Functioni
Inhibitor 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.
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).
Miscellaneous
Sites
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Sitei | 352 – 353 | (Microbial infection) Cleavage; by Staphylococcus aureus aureolysin/Aur1 Publication | 2 | |
Sitei | 354 – 355 | (Microbial infection) Cleavage; by Staphylococcus aureus serine and cysteine proteinases1 Publication | 2 | |
Sitei | 382 – 383 | Reactive bond | 2 |
GO - Molecular functioni
- identical protein binding Source: IntAct
- protease binding Source: UniProtKB
- serine-type endopeptidase inhibitor activity Source: MGI
GO - Biological processi
- acute-phase response Source: UniProtKB-KW
- blood coagulation Source: UniProtKB-KW
- negative regulation of endopeptidase activity Source: GO_Central
Keywordsi
Molecular function | Protease inhibitor, Serine protease inhibitor |
Biological process | Acute phase, Blood coagulation, Hemostasis |
Enzyme and pathway databases
PathwayCommonsi | P01009 |
Reactomei | R-HSA-114608, Platelet degranulation R-HSA-204005, COPII-mediated vesicle transport R-HSA-381426, Regulation of Insulin-like Growth Factor (IGF) transport and uptake by Insulin-like Growth Factor Binding Proteins (IGFBPs) R-HSA-5694530, Cargo concentration in the ER R-HSA-6798695, Neutrophil degranulation R-HSA-8957275, Post-translational protein phosphorylation |
SignaLinki | P01009 |
SIGNORi | P01009 |
Protein family/group databases
MEROPSi | I04.001 |
Names & Taxonomyi
Protein namesi | Recommended name: Alpha-1-antitrypsinCuratedAlternative name(s): Alpha-1 protease inhibitor Alpha-1-antiproteinase Serpin A1 Cleaved into the following chain: |
Gene namesi | ORF Names:PRO0684, PRO2209 |
Organismi | Homo sapiens (Human) |
Taxonomic identifieri | 9606 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo |
Proteomesi |
|
Organism-specific databases
HGNCi | HGNC:8941, SERPINA1 |
MIMi | 107400, gene |
neXtProti | NX_P01009 |
VEuPathDBi | HostDB:ENSG00000197249 |
Subcellular locationi
Endoplasmic reticulum
Extracellular region or secreted
Note: The S and Z allele are not secreted effectively and accumulate intracellularly in the endoplasmic reticulum.
Extracellular region or secreted
Endoplasmic reticulum
- endoplasmic reticulum Source: UniProtKB
- endoplasmic reticulum lumen Source: Reactome
Extracellular region or secreted
- extracellular exosome Source: UniProtKB
- extracellular region Source: Reactome
- extracellular space Source: UniProtKB
Golgi apparatus
- Golgi apparatus Source: UniProtKB
Other locations
- collagen-containing extracellular matrix Source: BHF-UCL
- COPII-coated ER to Golgi transport vesicle Source: Reactome
- endoplasmic reticulum-Golgi intermediate compartment membrane Source: Reactome
- ficolin-1-rich granule lumen Source: Reactome
- intracellular membrane-bounded organelle Source: HPA
- platelet alpha granule lumen Source: Reactome
Keywords - Cellular componenti
Endoplasmic reticulum, Extracellular matrix, SecretedPathology & Biotechi
Involvement in diseasei
Alpha-1-antitrypsin deficiency (A1ATD)3 Publications
Mutagenesis
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Mutagenesisi | 382 | M → V: Oxidation-resistant inhibitor of therapeutic importance. 1 Publication | 1 |
Organism-specific databases
DisGeNETi | 5265 |
GeneReviewsi | SERPINA1 |
MalaCardsi | SERPINA1 |
MIMi | 613490, phenotype |
OpenTargetsi | ENSG00000197249 |
Orphaneti | 60, Alpha-1-antitrypsin deficiency 586, Cystic fibrosis 178396, Hemorrhagic disease due to alpha-1-antitrypsin Pittsburgh mutation |
PharmGKBi | PA35509 |
Miscellaneous databases
Pharosi | P01009, Tbio |
Chemistry databases
DrugBanki | DB01998, 2-[3,4-Dihydroxy-2-Hydroxymethyl-5-(2-Hydroxy-Nonyl)-Tetrahydro-Furan-2-Yloxy]-6-Hydroxymethyl-Tetra Hydro-Pyran-3,4,5-Triol DB09130, Copper DB00080, Daptomycin DB03345, Mercaptoethanol DB14007, Pentetic acid DB05961, PPL-100 DB05481, Recombinant alpha 1-antitrypsin DB01593, Zinc DB14487, Zinc acetate DB14533, Zinc chloride DB14548, Zinc sulfate, unspecified form |
Genetic variation databases
BioMutai | SERPINA1 |
DMDMi | 1703025 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Signal peptidei | 1 – 24 | 1 PublicationAdd BLAST | 24 | |
ChainiPRO_0000032377 | 25 – 418 | Alpha-1-antitrypsin1 PublicationAdd BLAST | 394 | |
PeptideiPRO_0000364030 | 375 – 418 | Short peptide from AATAdd BLAST | 44 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Modified residuei | 38 | Phosphoserine; by FAM20C1 Publication | 1 | |
Glycosylationi | 70 | N-linked (GlcNAc...) (complex) asparagine9 Publications | 1 | |
Glycosylationi | 107 | N-linked (GlcNAc...) (complex) asparagine5 Publications | 1 | |
Modified residuei | 256 | S-cysteinyl cysteine | 1 | |
Glycosylationi | 271 | N-linked (GlcNAc...) (complex) asparagine9 Publications | 1 | |
Modified residuei | 383 | PhosphoserineCombined sources | 1 |
Post-translational modificationi
Keywords - PTMi
Glycoprotein, PhosphoproteinProteomic databases
CPTACi | CPTAC-651 non-CPTAC-1063 |
EPDi | P01009 |
jPOSTi | P01009 |
MassIVEi | P01009 |
MaxQBi | P01009 |
PaxDbi | P01009 |
PeptideAtlasi | P01009 |
PRIDEi | P01009 |
ProteomicsDBi | 51300 [P01009-1] 51301 [P01009-2] 51302 [P01009-3] |
2D gel databases
DOSAC-COBS-2DPAGEi | P01009 |
OGPi | P01009 |
REPRODUCTION-2DPAGEi | IPI00553177 P01009 |
SWISS-2DPAGEi | P01009 |
UCD-2DPAGEi | P01009 |
PTM databases
CarbonylDBi | P01009 |
GlyConnecti | 20, 101 N-Linked glycans (4 sites), 3 O-Linked glycans (2 sites) |
GlyGeni | P01009, 11 sites, 112 N-linked glycans (5 sites), 5 O-linked glycans (6 sites) |
iPTMneti | P01009 |
MetOSitei | P01009 |
PhosphoSitePlusi | P01009 |
Expressioni
Tissue specificityi
Gene expression databases
Bgeei | ENSG00000197249, Expressed in liver and 220 other tissues |
ExpressionAtlasi | P01009, baseline and differential |
Genevisiblei | P01009, HS |
Organism-specific databases
HPAi | ENSG00000197249, Tissue enriched (liver) |
Interactioni
Subunit structurei
Binary interactionsi
P01009
GO - Molecular functioni
- identical protein binding Source: IntAct
- protease binding Source: UniProtKB
Protein-protein interaction databases
BioGRIDi | 111283, 153 interactors |
CORUMi | P01009 |
DIPi | DIP-35493N |
IntActi | P01009, 44 interactors |
MINTi | P01009 |
STRINGi | 9606.ENSP00000416066 |
Miscellaneous databases
RNActi | P01009, protein |
Structurei
Secondary structure
3D structure databases
AlphaFoldDBi | P01009 |
BMRBi | P01009 |
PCDDBi | P01009 |
SMRi | P01009 |
ModBasei | Search... |
PDBe-KBi | Search... |
Miscellaneous databases
EvolutionaryTracei | P01009 |
Family & Domainsi
Region
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Regioni | 368 – 392 | RCLAdd BLAST | 25 |
Domaini
Sequence similaritiesi
Keywords - Domaini
SignalPhylogenomic databases
eggNOGi | KOG2392, Eukaryota |
GeneTreei | ENSGT00940000154493 |
HOGENOMi | CLU_023330_2_1_1 |
InParanoidi | P01009 |
OMAi | MEAIPMS |
PhylomeDBi | P01009 |
TreeFami | TF343201 |
Family and domain databases
Gene3Di | 2.30.39.10, 1 hit 3.30.497.10, 1 hit |
InterProi | View protein in InterPro IPR023795, Serpin_CS IPR023796, Serpin_dom IPR000215, Serpin_fam IPR036186, Serpin_sf IPR042178, Serpin_sf_1 IPR042185, Serpin_sf_2 |
PANTHERi | PTHR11461, PTHR11461, 1 hit |
Pfami | View protein in Pfam PF00079, Serpin, 1 hit |
SMARTi | View protein in SMART SM00093, SERPIN, 1 hit |
SUPFAMi | SSF56574, SSF56574, 1 hit |
PROSITEi | View protein in PROSITE PS00284, SERPIN, 1 hit |
s (3+)i Sequence
Sequence statusi: Complete.
: The displayed sequence is further processed into a mature form. Sequence processingi
This entry describes 3 produced by isoformsialternative splicing. AlignAdd to basketThis entry has 3 described isoforms and 8 potential isoforms that are computationally mapped.Show allAlign All
This isoform has been chosen as the sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry. canonicali
10 20 30 40 50
MPSSVSWGIL LLAGLCCLVP VSLAEDPQGD AAQKTDTSHH DQDHPTFNKI
60 70 80 90 100
TPNLAEFAFS LYRQLAHQSN STNIFFSPVS IATAFAMLSL GTKADTHDEI
110 120 130 140 150
LEGLNFNLTE IPEAQIHEGF QELLRTLNQP DSQLQLTTGN GLFLSEGLKL
160 170 180 190 200
VDKFLEDVKK LYHSEAFTVN FGDTEEAKKQ INDYVEKGTQ GKIVDLVKEL
210 220 230 240 250
DRDTVFALVN YIFFKGKWER PFEVKDTEEE DFHVDQVTTV KVPMMKRLGM
260 270 280 290 300
FNIQHCKKLS SWVLLMKYLG NATAIFFLPD EGKLQHLENE LTHDIITKFL
310 320 330 340 350
ENEDRRSASL HLPKLSITGT YDLKSVLGQL GITKVFSNGA DLSGVTEEAP
360 370 380 390 400
LKLSKAVHKA VLTIDEKGTE AAGAMFLEAI PMSIPPEVKF NKPFVFLMIE
410
QNTKSPLFMG KVVNPTQK
Computationally mapped potential isoform sequencesi
There are 8 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basketA0A024R6I7 | A0A024R6I7_HUMAN | Alpha-1-antitrypsin | SERPINA1 hCG_2029168 | 418 | Annotation score: | ||
A0A0G2JRN3 | A0A0G2JRN3_HUMAN | Alpha-1-antitrypsin | SERPINA1 | 359 | Annotation score: | ||
G3V387 | G3V387_HUMAN | Alpha-1-antitrypsin | SERPINA1 | 117 | Annotation score: | ||
G3V544 | G3V544_HUMAN | Alpha-1-antitrypsin | SERPINA1 | 156 | Annotation score: | ||
G3V5R8 | G3V5R8_HUMAN | Alpha-1-antitrypsin | SERPINA1 | 92 | Annotation score: | ||
A0A0B4J278 | A0A0B4J278_HUMAN | Alpha-1-antitrypsin | SERPINA1 | 144 | Annotation score: | ||
G3V2B9 | G3V2B9_HUMAN | Alpha-1-antitrypsin | SERPINA1 | 105 | Annotation score: | ||
G3V4I7 | G3V4I7_HUMAN | Alpha-1-antitrypsin | SERPINA1 | 22 | Annotation score: |
Sequence cautioni
Experimental Info
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Sequence conflicti | 12 | Missing in AAA51546 (PubMed:2985281).Curated | 1 | |
Sequence conflicti | 23 | L → P in BAG38005 (PubMed:14702039).Curated | 1 | |
Sequence conflicti | 26 | D → H AA sequence (PubMed:1906855).Curated | 1 | |
Sequence conflicti | 39 | H → L AA sequence (PubMed:1906855).Curated | 1 | |
Sequence conflicti | 61 | L → P in AAF29581 (Ref. 9) Curated | 1 | |
Sequence conflicti | 96 | T → A in ABG73380 (PubMed:17650587).Curated | 1 | |
Sequence conflicti | 139 – 140 | GN → DG in AAB59375 (PubMed:6319097).Curated | 2 | |
Sequence conflicti | 174 | T → H in AAA51546 (PubMed:2985281).Curated | 1 | |
Sequence conflicti | 229 | E → D in AAA51546 (PubMed:2985281).Curated | 1 | |
Sequence conflicti | 273 | T → N in AAB59375 (PubMed:6319097).Curated | 1 | |
Sequence conflicti | 280 | D → G in ABG73380 (PubMed:17650587).Curated | 1 | |
Sequence conflicti | 326 | V → I in CAA25838 (PubMed:6387509).Curated | 1 | |
Sequence conflicti | 410 | G → L AA sequence (PubMed:1406456).Curated | 1 | |
Sequence conflicti | 414 | N → S AA sequence (PubMed:1406456).Curated | 1 |
Polymorphismi
Natural variant
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_006978 | 4 | S → L in Z-Wrexham. 1 Publication | 1 | |
Natural variantiVAR_006979 | 26 | D → A in V-Munich. 1 PublicationCorresponds to variant dbSNP:rs199422212EnsemblClinVar. | 1 | |
Natural variantiVAR_051938 | 37 | T → A. Corresponds to variant dbSNP:rs11558262Ensembl. | 1 | |
Natural variantiVAR_006980 | 58 | A → T in M5-Karlsruhe. 1 PublicationCorresponds to variant dbSNP:rs149319176Ensembl. | 1 | |
Natural variantiVAR_006981 | 63 | R → C in I. 1 PublicationCorresponds to variant dbSNP:rs28931570EnsemblClinVar. | 1 | |
Natural variantiVAR_006982 | 65 | L → P in M-Procida. 1 PublicationCorresponds to variant dbSNP:rs28931569EnsemblClinVar. | 1 | |
Natural variantiVAR_006983 | 69 | S → F in M6-Bonn. 1 PublicationCorresponds to variant dbSNP:rs199687431EnsemblClinVar. | 1 | |
Natural variantiVAR_006984 | 75 | Missing in M-Malton, M-Nichinan and M-Palermo; associated with very low serum levels of AAT; homozygosity for allele M-Malton may be associated with a risk for chronic emphysema or infantile liver cirrhosis. 4 Publications | 1 | |
Natural variantiVAR_006985 | 77 | S → F in S-Iiyama. 1 PublicationCorresponds to variant dbSNP:rs55819880EnsemblClinVar. | 1 | |
Natural variantiVAR_006986 | 84 | A → T in M6-Passau. 1 PublicationCorresponds to variant dbSNP:rs111850950EnsemblClinVar. | 1 | |
Natural variantiVAR_006987 | 91 | G → E in M-Mineral springs; causes reduced AAT secretion. 1 PublicationCorresponds to variant dbSNP:rs28931568EnsemblClinVar. | 1 | |
Natural variantiVAR_006988 | 92 | T → I in QO-Lisbon; deficient AAT with very low serum levels. Corresponds to variant dbSNP:rs1490133295Ensembl. | 1 | |
Natural variantiVAR_011620 | 109 | T → M in Z-Bristol; deficient AA; disrupts the N-glycosylation site N-107. 1 PublicationCorresponds to variant dbSNP:rs199422213EnsemblClinVar. | 1 | |
Natural variantiVAR_006989 | 112 | P → T in M5-Berlin. 1 PublicationCorresponds to variant dbSNP:rs886044322EnsemblClinVar. | 1 | |
Natural variantiVAR_006990 | 116 | I → N in QO-Ludwigshafen. 1 PublicationCorresponds to variant dbSNP:rs28931572EnsemblClinVar. | 1 | |
Natural variantiVAR_006991 | 125 | R → H in M2; associated with D-400. Corresponds to variant dbSNP:rs709932EnsemblClinVar. | 1 | |
Natural variantiVAR_006992 | 139 | G → S in QO-Newport. 1 PublicationCorresponds to variant dbSNP:rs11558261EnsemblClinVar. | 1 | |
Natural variantiVAR_006993 | 172 | G → R in V and M-Nichinan. 1 PublicationCorresponds to variant dbSNP:rs112030253EnsemblClinVar. | 1 | |
Natural variantiVAR_006994 | 172 | G → W in M2-Obernburg. 2 PublicationsCorresponds to variant dbSNP:rs112030253EnsemblClinVar. | 1 | |
Natural variantiVAR_006995 | 180 | Q → E in L-Frankfurt. 1 PublicationCorresponds to variant dbSNP:rs864622051EnsemblClinVar. | 1 | |
Natural variantiVAR_036746 | 190 – 198 | QGKIVDLVK → GFQNAILVR in Aberrant form. | 9 | |
Natural variantiVAR_006996 | 228 | E → K in X. Corresponds to variant dbSNP:rs199422208EnsemblClinVar. | 1 | |
Natural variantiVAR_006997 | 237 | V → A in M1A and Z; associated with K-366 in Z. 4 PublicationsCorresponds to variant dbSNP:rs6647EnsemblClinVar. | 1 | |
Natural variantiVAR_006998 | 247 | R → C in F. 1 PublicationCorresponds to variant dbSNP:rs28929470EnsemblClinVar. | 1 | |
Natural variantiVAR_006999 | 280 | D → V in P-Duarte/P-Cardiff/P-Lowell; associated with H-415 in Y-Barcelona. 3 PublicationsCorresponds to variant dbSNP:rs121912714EnsemblClinVar. | 1 | |
Natural variantiVAR_007000 | 288 | E → V in S and T. 2 PublicationsCorresponds to variant dbSNP:rs17580EnsemblClinVar. | 1 | |
Natural variantiVAR_009216 | 305 | Missing in Basque. 1 Publication | 1 | |
Natural variantiVAR_007001 | 354 | S → F in S-Munich. 1 PublicationCorresponds to variant dbSNP:rs201788603EnsemblClinVar. | 1 | |
Natural variantiVAR_007002 | 360 | A → T in W-Bethesda. 1 PublicationCorresponds to variant dbSNP:rs1802959EnsemblClinVar. | 1 | |
Natural variantiVAR_007003 | 365 | D → N in P-St.Albans/P-Donauwoerth. 1 PublicationCorresponds to variant dbSNP:rs143370956EnsemblClinVar. | 1 | |
Natural variantiVAR_007004 | 366 | E → K in Z/Z-Augsburg/Z-Tun; associated with A-237 in Z. 3 PublicationsCorresponds to variant dbSNP:rs28929474EnsemblClinVar. | 1 | |
Natural variantiVAR_007005 | 382 | M → R in Pittsburgh; has antithrombin activity; inhibits factor VIIa activity; causes fatal bleeding diathesis. 3 PublicationsCorresponds to variant dbSNP:rs121912713EnsemblClinVar. | 1 | |
Natural variantiVAR_007006 | 386 | P → H in Sao Tome. 1 PublicationCorresponds to variant dbSNP:rs569384943Ensembl. | 1 | |
Natural variantiVAR_007007 | 386 | P → T in L-Offenbach. 1 PublicationCorresponds to variant dbSNP:rs12233Ensembl. | 1 | |
Natural variantiVAR_007008 | 387 | E → K in Christchurch. Corresponds to variant dbSNP:rs121912712EnsemblClinVar. | 1 | |
Natural variantiVAR_007009 | 393 | P → L in M-Heerlen. 1 PublicationCorresponds to variant dbSNP:rs199422209EnsemblClinVar. | 1 | |
Natural variantiVAR_007010 | 400 | E → D in M2 and M3; associated with H-125 in M2. 2 PublicationsCorresponds to variant dbSNP:rs1303EnsemblClinVar. | 1 | |
Natural variantiVAR_007011 | 415 | P → H in Y-Barcelona; associated with V-280. 1 Publication | 1 |
Alternative sequence
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Alternative sequenceiVSP_028890 | 307 – 418 | Missing in isoform 3. 1 PublicationAdd BLAST | 112 | |
Alternative sequenceiVSP_028889 | 356 – 418 | AVHKA…NPTQK → VRSP in isoform 2. 1 PublicationAdd BLAST | 63 |
Sequence databases
Genome annotation databases
Keywords - Coding sequence diversityi
Alternative splicingSimilar proteinsi
Cross-referencesi
Web resourcesi
Wikipedia Alpha-1 antitrypsin entry |
Sequence databases
3D structure databases
Select the link destinations: PDBei RCSB PDBi PDBji Links Updated | PDB entry | Method | Resolution (Å) | Chain | Positions | PDBsum |
1ATU | X-ray | 2.70 | A | 45-418 | [»] | |
1D5S | X-ray | 3.00 | A | 44-377 | [»] | |
B | 378-418 | [»] | ||||
1EZX | X-ray | 2.60 | A | 48-382 | [»] | |
B | 383-418 | [»] | ||||
1HP7 | X-ray | 2.10 | A | 25-418 | [»] | |
1IZ2 | X-ray | 2.20 | A | 25-418 | [»] | |
1KCT | X-ray | 3.46 | A | 25-418 | [»] | |
1OO8 | X-ray | 2.65 | A | 26-418 | [»] | |
1OPH | X-ray | 2.30 | A | 26-418 | [»] | |
1PSI | X-ray | 2.92 | A | 26-418 | [»] | |
1QLP | X-ray | 2.00 | A | 26-418 | [»] | |
1QMB | X-ray | 2.60 | A | 49-376 | [»] | |
B | 377-418 | [»] | ||||
2D26 | X-ray | 3.30 | A | 25-382 | [»] | |
B | 383-418 | [»] | ||||
2QUG | X-ray | 2.00 | A | 25-418 | [»] | |
3CWL | X-ray | 2.44 | A | 25-418 | [»] | |
3CWM | X-ray | 2.51 | A | 25-418 | [»] | |
3DRM | X-ray | 2.20 | A | 26-418 | [»] | |
3DRU | X-ray | 3.20 | A/B/C | 26-418 | [»] | |
3NDD | X-ray | 1.50 | A | 46-382 | [»] | |
B | 383-418 | [»] | ||||
3NDF | X-ray | 2.70 | A | 46-382 | [»] | |
B | 383-418 | [»] | ||||
3NE4 | X-ray | 1.81 | A | 48-418 | [»] | |
3T1P | X-ray | 3.90 | A | 48-418 | [»] | |
4PYW | X-ray | 1.91 | A | 26-418 | [»] | |
5IO1 | X-ray | 3.34 | A/B | 29-418 | [»] | |
5NBU | X-ray | 1.67 | A | 43-418 | [»] | |
5NBV | X-ray | 1.73 | A | 43-418 | [»] | |
6HX4 | X-ray | 2.95 | A/B | 24-418 | [»] | |
6I4V | X-ray | 1.78 | A | 26-418 | [»] | |
6I7U | X-ray | 1.55 | A | 26-418 | [»] | |
6IAY | X-ray | 1.90 | A | 26-418 | [»] | |
6ROD | X-ray | 1.85 | A | 26-418 | [»] | |
7AEL | X-ray | 1.76 | AAA | 26-418 | [»] | |
7API | X-ray | 3.00 | A | 36-382 | [»] | |
B | 383-418 | [»] | ||||
7NPK | X-ray | 1.83 | A | 26-417 | [»] | |
7NPL | X-ray | 1.82 | A | 26-418 | [»] | |
8API | X-ray | 3.10 | A | 36-382 | [»] | |
B | 383-418 | [»] | ||||
9API | X-ray | 3.00 | A | 36-382 | [»] | |
B | 383-418 | [»] | ||||
AlphaFoldDBi | P01009 | |||||
BMRBi | P01009 | |||||
PCDDBi | P01009 | |||||
SMRi | P01009 | |||||
ModBasei | Search... | |||||
PDBe-KBi | Search... |
Protein-protein interaction databases
BioGRIDi | 111283, 153 interactors |
CORUMi | P01009 |
DIPi | DIP-35493N |
IntActi | P01009, 44 interactors |
MINTi | P01009 |
STRINGi | 9606.ENSP00000416066 |
Chemistry databases
DrugBanki | DB01998, 2-[3,4-Dihydroxy-2-Hydroxymethyl-5-(2-Hydroxy-Nonyl)-Tetrahydro-Furan-2-Yloxy]-6-Hydroxymethyl-Tetra Hydro-Pyran-3,4,5-Triol DB09130, Copper DB00080, Daptomycin DB03345, Mercaptoethanol DB14007, Pentetic acid DB05961, PPL-100 DB05481, Recombinant alpha 1-antitrypsin DB01593, Zinc DB14487, Zinc acetate DB14533, Zinc chloride DB14548, Zinc sulfate, unspecified form |
Protein family/group databases
MEROPSi | I04.001 |
PTM databases
CarbonylDBi | P01009 |
GlyConnecti | 20, 101 N-Linked glycans (4 sites), 3 O-Linked glycans (2 sites) |
GlyGeni | P01009, 11 sites, 112 N-linked glycans (5 sites), 5 O-linked glycans (6 sites) |
iPTMneti | P01009 |
MetOSitei | P01009 |
PhosphoSitePlusi | P01009 |
Genetic variation databases
BioMutai | SERPINA1 |
DMDMi | 1703025 |
2D gel databases
DOSAC-COBS-2DPAGEi | P01009 |
OGPi | P01009 |
REPRODUCTION-2DPAGEi | IPI00553177 P01009 |
SWISS-2DPAGEi | P01009 |
UCD-2DPAGEi | P01009 |
Proteomic databases
CPTACi | CPTAC-651 non-CPTAC-1063 |
EPDi | P01009 |
jPOSTi | P01009 |
MassIVEi | P01009 |
MaxQBi | P01009 |
PaxDbi | P01009 |
PeptideAtlasi | P01009 |
PRIDEi | P01009 |
ProteomicsDBi | 51300 [P01009-1] 51301 [P01009-2] 51302 [P01009-3] |
Protocols and materials databases
ABCDi | P01009, 3 sequenced antibodies |
Antibodypediai | 767, 1693 antibodies from 48 providers |
CPTCi | P01009, 1 antibody |
DNASUi | 5265 |
Genome annotation databases
Organism-specific databases
CTDi | 5265 |
DisGeNETi | 5265 |
GeneCardsi | SERPINA1 |
GeneReviewsi | SERPINA1 |
HGNCi | HGNC:8941, SERPINA1 |
HPAi | ENSG00000197249, Tissue enriched (liver) |
MalaCardsi | SERPINA1 |
MIMi | 107400, gene 613490, phenotype |
neXtProti | NX_P01009 |
OpenTargetsi | ENSG00000197249 |
Orphaneti | 60, Alpha-1-antitrypsin deficiency 586, Cystic fibrosis 178396, Hemorrhagic disease due to alpha-1-antitrypsin Pittsburgh mutation |
PharmGKBi | PA35509 |
VEuPathDBi | HostDB:ENSG00000197249 |
GenAtlasi | Search... |
Phylogenomic databases
eggNOGi | KOG2392, Eukaryota |
GeneTreei | ENSGT00940000154493 |
HOGENOMi | CLU_023330_2_1_1 |
InParanoidi | P01009 |
OMAi | MEAIPMS |
PhylomeDBi | P01009 |
TreeFami | TF343201 |
Enzyme and pathway databases
PathwayCommonsi | P01009 |
Reactomei | R-HSA-114608, Platelet degranulation R-HSA-204005, COPII-mediated vesicle transport R-HSA-381426, Regulation of Insulin-like Growth Factor (IGF) transport and uptake by Insulin-like Growth Factor Binding Proteins (IGFBPs) R-HSA-5694530, Cargo concentration in the ER R-HSA-6798695, Neutrophil degranulation R-HSA-8957275, Post-translational protein phosphorylation |
SignaLinki | P01009 |
SIGNORi | P01009 |
Miscellaneous databases
BioGRID-ORCSi | 5265, 9 hits in 1081 CRISPR screens |
ChiTaRSi | SERPINA1, human |
EvolutionaryTracei | P01009 |
GeneWikii | Alpha_1-antitrypsin |
GenomeRNAii | 5265 |
Pharosi | P01009, Tbio |
PROi | PR:P01009 |
RNActi | P01009, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSG00000197249, Expressed in liver and 220 other tissues |
ExpressionAtlasi | P01009, baseline and differential |
Genevisiblei | P01009, HS |
Family and domain databases
Gene3Di | 2.30.39.10, 1 hit 3.30.497.10, 1 hit |
InterProi | View protein in InterPro IPR023795, Serpin_CS IPR023796, Serpin_dom IPR000215, Serpin_fam IPR036186, Serpin_sf IPR042178, Serpin_sf_1 IPR042185, Serpin_sf_2 |
PANTHERi | PTHR11461, PTHR11461, 1 hit |
Pfami | View protein in Pfam PF00079, Serpin, 1 hit |
SMARTi | View protein in SMART SM00093, SERPIN, 1 hit |
SUPFAMi | SSF56574, SSF56574, 1 hit |
PROSITEi | View protein in PROSITE PS00284, SERPIN, 1 hit |
MobiDBi | Search... |
Entry informationi
Entry namei | A1AT_HUMAN | |
Accessioni | P01009Primary (citable) accession number: P01009 Secondary accession number(s): A6PX14 Q9UCM3 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | July 21, 1986 |
Last sequence update: | October 1, 1996 | |
Last modified: | May 25, 2022 | |
This is version 273 of the entry and version 3 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program | |
Disclaimer | Any medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care. |
Miscellaneousi
Keywords - Technical termi
3D-structure, Direct protein sequencing, Reference proteomeDocuments
- Human chromosome 14
Human chromosome 14: entries, gene names and cross-references to MIM - Human entries with genetic variants
List of human entries with genetic variants - Human variants curated from literature reports
Index of human variants curated from literature reports - MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot - PDB cross-references
Index of Protein Data Bank (PDB) cross-references - SIMILARITY comments
Index of protein domains and families