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Protein

Alpha-1-antichymotrypsin

Gene

SERPINA3

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Although its physiological function is unclear, it can inhibit neutrophil cathepsin G and mast cell chymase, both of which can convert angiotensin-1 to the active angiotensin-2.1 Publication

Miscellaneous

Alpha-1-antichymotrypsin can bind DNA.

Caution

It is uncertain whether Met-1 or Met-4 is the initiator.Curated

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sitei383 – 384Reactive bond2

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
DNA bindingi235 – 2373

GO - Molecular functioni

  • DNA binding Source: UniProtKB
  • serine-type endopeptidase inhibitor activity Source: GO_Central

GO - Biological processi

Keywordsi

Molecular functionProtease inhibitor, Serine protease inhibitor
Biological processAcute phase

Enzyme and pathway databases

ReactomeiR-HSA-114608 Platelet degranulation
R-HSA-6798695 Neutrophil degranulation
SIGNORiP01011

Protein family/group databases

MEROPSiI04.002

Names & Taxonomyi

Protein namesi
Recommended name:
Alpha-1-antichymotrypsin
Short name:
ACT
Alternative name(s):
Cell growth-inhibiting gene 24/25 protein
Serpin A3
Cleaved into the following chain:
Gene namesi
Name:SERPINA3
Synonyms:AACT
ORF Names:GIG24, GIG25
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 14

Organism-specific databases

EuPathDBiHostDB:ENSG00000196136.16
HGNCiHGNC:16 SERPINA3
MIMi107280 gene
neXtProtiNX_P01011

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Secreted

Pathology & Biotechi

Keywords - Diseasei

Disease mutation

Organism-specific databases

DisGeNETi12
OpenTargetsiENSG00000196136
Orphaneti93594 Alpha-1-antichymotrypsin deficiency
PharmGKBiPA35020

Chemistry databases

ChEMBLiCHEMBL5960

Polymorphism and mutation databases

BioMutaiSERPINA3
DMDMi112874

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 231 PublicationAdd BLAST23
ChainiPRO_000003241124 – 423Alpha-1-antichymotrypsinAdd BLAST400
ChainiPRO_000003241226 – 423Alpha-1-antichymotrypsin His-Pro-lessAdd BLAST398

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Glycosylationi33N-linked (GlcNAc...) asparagine1 Publication1
Glycosylationi93N-linked (GlcNAc...) asparagine4 Publications1
Glycosylationi106N-linked (GlcNAc...) asparagine3 Publications1
Glycosylationi127N-linked (GlcNAc...) asparagine2 Publications1
Glycosylationi186N-linked (GlcNAc...) asparagine1 Publication1
Glycosylationi271N-linked (GlcNAc...) asparagine2 Publications1

Post-translational modificationi

N- and O-glycosylated.5 Publications

Keywords - PTMi

Glycoprotein

Proteomic databases

EPDiP01011
MaxQBiP01011
PaxDbiP01011
PeptideAtlasiP01011
PRIDEiP01011
ProteomicsDBi51303
51304 [P01011-2]
51305 [P01011-3]

2D gel databases

DOSAC-COBS-2DPAGEiP01011
SWISS-2DPAGEiP01011

PTM databases

CarbonylDBiP01011
GlyConnecti19
iPTMnetiP01011
PhosphoSitePlusiP01011
UniCarbKBiP01011

Miscellaneous databases

PMAP-CutDBiP01011

Expressioni

Tissue specificityi

Plasma. Synthesized in the liver. Like the related alpha-1-antitrypsin, its concentration increases in the acute phase of inflammation or infection. Found in the amyloid plaques from the hippocampus of Alzheimer disease brains.2 Publications

Gene expression databases

BgeeiENSG00000196136 Expressed in 92 organ(s), highest expression level in liver
ExpressionAtlasiP01011 baseline and differential
GenevisibleiP01011 HS

Organism-specific databases

HPAiCAB016647
HPA000893
HPA002560

Interactioni

Subunit structurei

Interacts with DNAJC1.1 Publication

Binary interactionsi

WithEntry#Exp.IntActNotes
DNAJC1Q96KC83EBI-296557,EBI-296550

Protein-protein interaction databases

BioGridi106530, 29 interactors
CORUMiP01011
IntActiP01011, 10 interactors
STRINGi9606.ENSP00000376793

Structurei

Secondary structure

1423
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

ProteinModelPortaliP01011
SMRiP01011
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiP01011

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni369 – 394RCLAdd BLAST26
Regioni381 – 389O-glycosylated at one site9

Domaini

The reactive center loop (RCL) extends out from the body of the protein and directs binding to the target protease. The protease cleaves the serpin at the reactive site within the RCL, establishing a covalent linkage between the carboxyl group of the serpin reactive site and the serine hydroxyl of the protease. The resulting inactive serpin-protease complex is highly stable.

Sequence similaritiesi

Belongs to the serpin family.Curated

Keywords - Domaini

Signal

Phylogenomic databases

eggNOGiKOG2392 Eukaryota
COG4826 LUCA
GeneTreeiENSGT00760000118839
HOVERGENiHBG005957
InParanoidiP01011
KOiK04525
OMAiFLMIIVP
OrthoDBiEOG091G0ION
PhylomeDBiP01011
TreeFamiTF343201

Family and domain databases

InterProiView protein in InterPro
IPR023795 Serpin_CS
IPR023796 Serpin_dom
IPR000215 Serpin_fam
IPR036186 Serpin_sf
PANTHERiPTHR11461 PTHR11461, 1 hit
PfamiView protein in Pfam
PF00079 Serpin, 1 hit
SMARTiView protein in SMART
SM00093 SERPIN, 1 hit
SUPFAMiSSF56574 SSF56574, 1 hit
PROSITEiView protein in PROSITE
PS00284 SERPIN, 1 hit

Sequences (3+)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 3 isoformsi produced by alternative splicing. AlignAdd to basket

This entry has 3 described isoforms and 3 potential isoforms that are computationally mapped.Show allAlign All

Isoform 1 (identifier: P01011-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide
        10         20         30         40         50
MERMLPLLAL GLLAAGFCPA VLCHPNSPLD EENLTQENQD RGTHVDLGLA
60 70 80 90 100
SANVDFAFSL YKQLVLKAPD KNVIFSPLSI STALAFLSLG AHNTTLTEIL
110 120 130 140 150
KGLKFNLTET SEAEIHQSFQ HLLRTLNQSS DELQLSMGNA MFVKEQLSLL
160 170 180 190 200
DRFTEDAKRL YGSEAFATDF QDSAAAKKLI NDYVKNGTRG KITDLIKDLD
210 220 230 240 250
SQTMMVLVNY IFFKAKWEMP FDPQDTHQSR FYLSKKKWVM VPMMSLHHLT
260 270 280 290 300
IPYFRDEELS CTVVELKYTG NASALFILPD QDKMEEVEAM LLPETLKRWR
310 320 330 340 350
DSLEFREIGE LYLPKFSISR DYNLNDILLQ LGIEEAFTSK ADLSGITGAR
360 370 380 390 400
NLAVSQVVHK AVLDVFEEGT EASAATAVKI TLLSALVETR TIVRFNRPFL
410 420
MIIVPTDTQN IFFMSKVTNP KQA
Length:423
Mass (Da):47,651
Last modified:August 1, 1991 - v2
Checksum:iB002F946C86A8951
GO
Isoform 2 (identifier: P01011-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     215-216: AK → ER
     217-423: Missing.

Note: No experimental confirmation available.
Show »
Length:216
Mass (Da):24,034
Checksum:i49312EDF91439D4D
GO
Isoform 3 (identifier: P01011-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     64-95: LVLKAPDKNVIFSPLSISTALAFLSLGAHNTT → SPRWSIRLCLMYLRRAQKHLLPQQSKSPSFLH
     96-423: Missing.

Note: No experimental confirmation available.
Show »
Length:95
Mass (Da):10,717
Checksum:i15B1759843F9809B
GO

Computationally mapped potential isoform sequencesi

There are 3 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
G3V595G3V595_HUMAN
Alpha-1-antichymotrypsin
SERPINA3
305Annotation score:
G3V3A0G3V3A0_HUMAN
Alpha-1-antichymotrypsin
SERPINA3 hCG_2039549
205Annotation score:
A0A087WY93A0A087WY93_HUMAN
Alpha-1-antichymotrypsin
SERPINA3
46Annotation score:

Sequence cautioni

The sequence AAA51543 differs from that shown. Reason: Frameshift at positions 101, 106, 111, 117, 123, 129 and 421.Curated
The sequence AAT08029 differs from that shown. Reason: Frameshift at position 4.Curated
The sequence AAT08029 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.Curated
The sequence BAD92297 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.Curated
The sequence CAA48671 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti55D → S AA sequence (PubMed:6687683).Curated1
Sequence conflicti69P → L in AAA51543 (PubMed:6606438).Curated1
Sequence conflicti101K → R in BAD92297 (Ref. 5) Curated1
Sequence conflicti106N → Y in AAT08028 (Ref. 3) Curated1
Sequence conflicti198D → N in AAT08029 (Ref. 3) Curated1
Sequence conflicti199L → P in AAA51543 (PubMed:6606438).Curated1
Sequence conflicti234S → N in AAT08029 (Ref. 3) Curated1
Sequence conflicti339S → G in AAT08028 (Ref. 3) Curated1
Sequence conflicti346I → S in AAT08028 (Ref. 3) Curated1
Sequence conflicti361 – 363AVL → VVS in AAA51543 (PubMed:6606438).Curated3

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_0069739A → T4 PublicationsCorresponds to variant dbSNP:rs4934EnsemblClinVar.1
Natural variantiVAR_00697478L → P in Bochum-1. 1 PublicationCorresponds to variant dbSNP:rs1800463EnsemblClinVar.1
Natural variantiVAR_006975167A → G. 1
Natural variantiVAR_006976252P → A in Bonn-1. 2 PublicationsCorresponds to variant dbSNP:rs17473EnsemblClinVar.1
Natural variantiVAR_037902267K → R1 PublicationCorresponds to variant dbSNP:rs17853314Ensembl.1
Natural variantiVAR_006977401M → V Associated with occlusive-cerebrovascular disease; Isehara-1. 2 PublicationsCorresponds to variant dbSNP:rs755521612Ensembl.1
Natural variantiVAR_011742407D → G. Corresponds to variant dbSNP:rs10956Ensembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_01422564 – 95LVLKA…AHNTT → SPRWSIRLCLMYLRRAQKHL LPQQSKSPSFLH in isoform 3. 1 PublicationAdd BLAST32
Alternative sequenceiVSP_01422696 – 423Missing in isoform 3. 1 PublicationAdd BLAST328
Alternative sequenceiVSP_014227215 – 216AK → ER in isoform 2. 1 Publication2
Alternative sequenceiVSP_014228217 – 423Missing in isoform 2. 1 PublicationAdd BLAST207

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
K01500 mRNA Translation: AAA51543.1 Frameshift.
X68733
, X68734, X68735, X68736, X68737 Genomic DNA Translation: CAA48671.1 Different initiation.
AY513275 mRNA Translation: AAT08028.1
AY513276 mRNA Translation: AAT08029.1 Sequence problems.
AK123091 mRNA Translation: BAG53869.1
AB209060 mRNA Translation: BAD92297.1 Different initiation.
BC003559 mRNA Translation: AAH03559.3
BC010530 mRNA Translation: AAH10530.1
BC013189 mRNA Translation: AAH13189.1
BC034554 mRNA Translation: AAH34554.1
BC070265 mRNA Translation: AAH70265.1
M18906 mRNA Translation: AAA51559.1
AF089747 mRNA Translation: AAD08810.1
J05176 mRNA Translation: AAA51560.1
X00947 Genomic DNA Translation: CAA25459.1
CCDSiCCDS32150.1 [P01011-1]
PIRiA90475 ITHUC
S62374
RefSeqiNP_001076.2, NM_001085.4 [P01011-1]
UniGeneiHs.534293
Hs.710488

Genome annotation databases

EnsembliENST00000393078; ENSP00000376793; ENSG00000196136 [P01011-1]
ENST00000393080; ENSP00000376795; ENSG00000196136 [P01011-1]
ENST00000467132; ENSP00000450540; ENSG00000196136 [P01011-1]
ENST00000556968; ENSP00000452476; ENSG00000196136 [P01011-2]
GeneIDi12
KEGGihsa:12
UCSCiuc001ydp.4 human [P01011-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar proteinsi

Cross-referencesi

Web resourcesi

Wikipedia

Alpha-1 antichymotrypsin entry

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
K01500 mRNA Translation: AAA51543.1 Frameshift.
X68733
, X68734, X68735, X68736, X68737 Genomic DNA Translation: CAA48671.1 Different initiation.
AY513275 mRNA Translation: AAT08028.1
AY513276 mRNA Translation: AAT08029.1 Sequence problems.
AK123091 mRNA Translation: BAG53869.1
AB209060 mRNA Translation: BAD92297.1 Different initiation.
BC003559 mRNA Translation: AAH03559.3
BC010530 mRNA Translation: AAH10530.1
BC013189 mRNA Translation: AAH13189.1
BC034554 mRNA Translation: AAH34554.1
BC070265 mRNA Translation: AAH70265.1
M18906 mRNA Translation: AAA51559.1
AF089747 mRNA Translation: AAD08810.1
J05176 mRNA Translation: AAA51560.1
X00947 Genomic DNA Translation: CAA25459.1
CCDSiCCDS32150.1 [P01011-1]
PIRiA90475 ITHUC
S62374
RefSeqiNP_001076.2, NM_001085.4 [P01011-1]
UniGeneiHs.534293
Hs.710488

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
1AS4X-ray2.10A43-383[»]
B387-423[»]
1QMNX-ray2.27A26-423[»]
2ACHX-ray2.70A24-383[»]
B384-423[»]
3CAAX-ray2.40A43-383[»]
B387-423[»]
3DLWX-ray2.70A25-423[»]
4CAAX-ray2.90A43-383[»]
B387-423[»]
ProteinModelPortaliP01011
SMRiP01011
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi106530, 29 interactors
CORUMiP01011
IntActiP01011, 10 interactors
STRINGi9606.ENSP00000376793

Chemistry databases

ChEMBLiCHEMBL5960

Protein family/group databases

MEROPSiI04.002

PTM databases

CarbonylDBiP01011
GlyConnecti19
iPTMnetiP01011
PhosphoSitePlusiP01011
UniCarbKBiP01011

Polymorphism and mutation databases

BioMutaiSERPINA3
DMDMi112874

2D gel databases

DOSAC-COBS-2DPAGEiP01011
SWISS-2DPAGEiP01011

Proteomic databases

EPDiP01011
MaxQBiP01011
PaxDbiP01011
PeptideAtlasiP01011
PRIDEiP01011
ProteomicsDBi51303
51304 [P01011-2]
51305 [P01011-3]

Protocols and materials databases

DNASUi12
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000393078; ENSP00000376793; ENSG00000196136 [P01011-1]
ENST00000393080; ENSP00000376795; ENSG00000196136 [P01011-1]
ENST00000467132; ENSP00000450540; ENSG00000196136 [P01011-1]
ENST00000556968; ENSP00000452476; ENSG00000196136 [P01011-2]
GeneIDi12
KEGGihsa:12
UCSCiuc001ydp.4 human [P01011-1]

Organism-specific databases

CTDi12
DisGeNETi12
EuPathDBiHostDB:ENSG00000196136.16
GeneCardsiSERPINA3
H-InvDBiHIX0079611
HGNCiHGNC:16 SERPINA3
HPAiCAB016647
HPA000893
HPA002560
MIMi107280 gene
neXtProtiNX_P01011
OpenTargetsiENSG00000196136
Orphaneti93594 Alpha-1-antichymotrypsin deficiency
PharmGKBiPA35020
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG2392 Eukaryota
COG4826 LUCA
GeneTreeiENSGT00760000118839
HOVERGENiHBG005957
InParanoidiP01011
KOiK04525
OMAiFLMIIVP
OrthoDBiEOG091G0ION
PhylomeDBiP01011
TreeFamiTF343201

Enzyme and pathway databases

ReactomeiR-HSA-114608 Platelet degranulation
R-HSA-6798695 Neutrophil degranulation
SIGNORiP01011

Miscellaneous databases

ChiTaRSiSERPINA3 human
EvolutionaryTraceiP01011
GeneWikiiAlpha_1-antichymotrypsin
GenomeRNAii12
PMAP-CutDBiP01011
PROiPR:P01011
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000196136 Expressed in 92 organ(s), highest expression level in liver
ExpressionAtlasiP01011 baseline and differential
GenevisibleiP01011 HS

Family and domain databases

InterProiView protein in InterPro
IPR023795 Serpin_CS
IPR023796 Serpin_dom
IPR000215 Serpin_fam
IPR036186 Serpin_sf
PANTHERiPTHR11461 PTHR11461, 1 hit
PfamiView protein in Pfam
PF00079 Serpin, 1 hit
SMARTiView protein in SMART
SM00093 SERPIN, 1 hit
SUPFAMiSSF56574 SSF56574, 1 hit
PROSITEiView protein in PROSITE
PS00284 SERPIN, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiAACT_HUMAN
AccessioniPrimary (citable) accession number: P01011
Secondary accession number(s): B3KVQ7
, Q13703, Q2TU87, Q2TU88, Q59GP9, Q6LBY8, Q6LDT7, Q6NSC9, Q8N177, Q96DW8, Q9UC47, Q9UNU9
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 21, 1986
Last sequence update: August 1, 1991
Last modified: November 7, 2018
This is version 223 of the entry and version 2 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny 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, Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  6. Human chromosome 14
    Human chromosome 14: entries, gene names and cross-references to MIM
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