ACVR1_HUMAN » Activin receptor type-1

ACVR1_HUMAN » Activin receptor type-1
Magnify ACVR1_HUMAN » Activin receptor type-1Enlarged view of image
3D view in GLMol or JMol

gray dot

Download Coordinates

gray dot

Topology in Plasma membrane
Topologyextracellular side
cytoplasmic side
ACVR1_HUMAN » Activin receptor type-1 » Activin receptor type I;ACTR-I; Activin receptor-like kinase 2;ALK-2; Serine/threonine-protein kinase receptor R1;SKR1; TGF-B superfamily receptor type I;TSR-I;
Hydrophobic Thickness 34.0 ± 2.2 Å
Tilt Angle 41 ± 3°
ΔGtransfer -27.2 kcal/mol
ΔGfold -19.9 kcal/mol
Links UniProtKB, Pfam, Interpro, iHOP, STRING, HGNC, HMDB
Topology Out
TM Segments 122-150 (122-152)
Pathways

Cytokine-cytokine receptor interaction (KEGG)

TGF-beta signaling pathway (KEGG)

PDB 3mtf (201-499), 4bgg (201-499), 3q4u (A/B/C/D=201-499), 3h9r (A=172-499), 4c02 (A=172-499), 3oom (A=201-499), 4dym (A=201-499)
OPM none
Complexes

ACVR1:ACVR1_HUMAN

Interactions

ACV1B, Complex: ACVR1:AVR2A:ACV1B, PubMed

ACVR1, Complex: Homotetramer of activin receptor type-1, PDBID: 3Q4U

AVR2A, Complex: AVR2B:ACVR1:AVR2A, PubMed

AVR2A, Complex: ACVR1:AVR2A, PubMed

AVR2B, Complex: ACVR1:AVR2B

BAMBI, Complex: ACVR1:BAMBI, PubMed

BMPR2, Complex: BMPR2:ACVR1, PubMed

BMR1A, Complex: ACVR1:BMR1A

EGLN, Complex: EGLN:ACVR1, PubMed

STX8, Complex: STX8:ACVR1, PubMed

TGFR1, Complex: TGFR1:ACVR1, PubMed

TGFR2, Complex: TGFR2:ACVR1, PubMed

Domains

AA: 33-103, PDBID: 1ES7, Subunit B, Seq Identity:30%, Activin types I and II receptor domain

AA: 179-206, PDBID: 3H9R, Subunit A, Seq Identity:100%, Transforming growth factor beta type I GS-motif

AA: 208-495, PDBID: 3H9R, Subunit A, Seq Identity:100%, Protein kinase domain

UniProt annotation for ACVR1_HUMAN » Activin receptor type-1
FUNCTION: On ligand binding, forms a receptor complex consisting of two type II and two type I transmembrane serine/threonine kinases. Type II receptors phosphorylate and activate type I receptors which autophosphorylate, then bind and activate SMAD transcriptional regulators. Receptor for activin. May be involved for left-right pattern formation during embryogenesis (By similarity).

CATALYTIC ACTIVITY: ATP + [receptor-protein] = ADP + [receptor- protein] phosphate.

SUBUNIT: Interacts with FKBP1A. Interacts with FCHO1.

TISSUE SPECIFICITY: Expressed in normal parenchymal cells, endothelial cells, fibroblasts and tumor-derived epithelial cells.

DISEASE: Fibrodysplasia ossificans progressiva (FOP) OMIM: A rare autosomal dominant connective tissue disorder resulting in skeletal malformations and progressive extraskeletal ossification. Heterotopic ossification begins in childhood and can be induced by trauma or may occur without warning. Bone formation is episodic and progressive, leading to a debilitating ankylosis of all major joints of the axial and appendicular skeleton, rendering movement impossible. Note=The disease is caused by mutations affecting the gene represented in this entry.

UniProt features for ACVR1_HUMAN » Activin receptor type-1
SIGNAL 1 20 By similarity.
CHAIN 21 509 Activin receptor type-1.
DOMAIN 178 207 GS.
DOMAIN 208 502 Protein kinase.
ACT_SITE 336 336 Proton acceptor (By similarity).
Amino Acid Sequence for ACVR1_HUMAN » Activin receptor type-1
MVDGVMILPV LIMIALPSPS MEDEKPKVNP KLYMCVCEGL SCGNEDHCEG QQCFSSLSIN DGFHVYQKGC FQVYEQGKMT CKTPPSPGQA VECCQGDWCN RNITAQLPTK GKSFPGTQNF HLEVGLIILS VVFAVCLLAC LLGVALRKFK RRNQERLNPR DVEYGTIEGL ITTNVGDSTL ADLLDHSCTS GSGSGLPFLV QRTVARQITL LECVGKGRYG EVWRGSWQGE NVAVKIFSSR DEKSWFRETE LYNTVMLRHE NILGFIASDM TSRHSSTQLW LITHYHEMGS LYDYLQLTTL DTVSCLRIVL SIASGLAHLH IEIFGTQGKP AIAHRDLKSK NILVKKNGQC CIADLGLAVM HSQSTNQLDV GNNPRVGTKR YMAPEVLDET IQVDCFDSYK RVDIWAFGLV LWEVARRMVS NGIVEDYKPP FYDVVPNDPS FEDMRKVVCV DQQRPNIPNR WFSDPTLTSL AKLMKECWYQ NPSARLTALR IKKTLTKIDN SLDKLKTDC