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Identification
HMDB Protein ID HMDBP07788
Secondary Accession Numbers
  • 13497
Name Mannan-binding lectin serine protease 2
Synonyms
  1. MASP-2
  2. MBL-associated serine protease 2
  3. Mannan-binding lectin serine protease 2 A chain
  4. Mannan-binding lectin serine protease 2 B chain
  5. Mannose-binding protein-associated serine protease 2
Gene Name MASP2
Protein Type Unknown
Biological Properties
General Function Involved in calcium ion binding
Specific Function Serum protease that plays an important role in the activation of the complement system via mannose-binding lectin. After activation by auto-catalytic cleavage it cleaves C2 and C4, leading to their activation and to the formation of C3 convertase
Pathways Not Available
Reactions Not Available
GO Classification
Function
endopeptidase activity
serine-type endopeptidase activity
ion binding
cation binding
metal ion binding
binding
catalytic activity
hydrolase activity
peptidase activity
peptidase activity, acting on l-amino acid peptides
calcium ion binding
Process
metabolic process
macromolecule metabolic process
protein metabolic process
proteolysis
Cellular Location
  1. Secreted
Gene Properties
Chromosome Location Chromosome:1
Locus 1p36.3-p36.2
SNPs MASP2
Gene Sequence
>2061 bp
ATGAGGCTGCTGACCCTCCTGGGCCTTCTGTGTGGCTCGGTGGCCACCCCCTTAGGCCCG
AAGTGGCCTGAACCTGTGTTCGGGCGCCTGGCATCCCCCGGCTTTCCAGGGGAGTATGCC
AATGACCAGGAGCGGCGCTGGACCCTGACTGCACCCCCCGGCTACCGCCTGCGCCTCTAC
TTCACCCACTTCGACCTGGAGCTCTCCCACCTCTGCGAGTACGACTTCGTCAAGCTGAGC
TCGGGGGCCAAGGTGCTGGCCACGCTGTGCGGGCAGGAGAGCACAGACACGGAGCGGGCC
CCTGGCAAGGACACTTTCTACTCGCTGGGCTCCAGCCTGGACATTACCTTCCGCTCCGAC
TACTCCAACGAGAAGCCGTTCACGGGGTTCGAGGCCTTCTATGCAGCCGAGGACATTGAC
GAGTGCCAGGTGGCCCCGGGAGAGGCGCCCACCTGCGACCACCACTGCCACAACCACCTG
GGCGGTTTCTACTGCTCCTGCCGCGCAGGCTACGTCCTGCACCGTAACAAGCGCACCTGC
TCAGCCCTGTGCTCCGGCCAGGTCTTCACCCAGAGGTCTGGGGAGCTCAGCAGCCCTGAA
TACCCACGGCCGTATCCCAAACTCTCCAGTTGCACTTACAGCATCAGCCTGGAGGAGGGG
TTCAGTGTCATTCTGGACTTTGTGGAGTCCTTCGATGTGGAGACACACCCTGAAACCCTG
TGTCCCTACGACTTTCTCAAGATTCAAACAGACAGAGAAGAACATGGCCCATTCTGTGGG
AAGACATTGCCCCACAGGATTGAAACAAAAAGCAACACGGTGACCATCACCTTTGTCACA
GATGAATCAGGAGACCACACAGGCTGGAAGATCCACTACACGAGCACAGCGCACGCTTGC
CCTTATCCGATGGCGCCACCTAATGGCCACGTTTCACCTGTGCAAGCCAAATACATCCTG
AAAGACAGCTTCTCCATCTTTTGCGAGACTGGCTATGAGCTTCTGCAAGGTCACTTGCCC
CTGAAATCCTTTACTGCAGTTTGTCAGAAAGATGGATCTTGGGACCGGCCAATGCCCGCG
TGCAGCATTGTTGACTGTGGCCCTCCTGATGATCTACCCAGTGGCCGAGTGGAGTACATC
ACAGGTCCTGGAGTGACCACCTACAAAGCTGTGATTCAGTACAGCTGTGAAGAGACCTTC
TACACAATGAAAGTGAATGATGGTAAATATGTGTGTGAGGCTGATGGATTCTGGACGAGC
TCCAAAGGAGAAAAATCACTCCCAGTCTGTGAGCCTGTTTGTGGACTATCAGCCCGCACA
ACAGGAGGGCGTATATATGGAGGGCAAAAGGCAAAACCTGGTGATTTTCCTTGGCAAGTC
CTGATATTAGGTGGAACCACAGCAGCAGGTGCACTTTTATATGACAACTGGGTCCTAACA
GCTGCTCATGCCGTCTATGAGCAAAAACATGATGCATCCGCCCTGGACATTCGAATGGGC
ACCCTGAAAAGACTATCACCTCATTATACACAAGCCTGGTCTGAAGCTGTTTTTATACAT
GAAGGTTATACTCATGATGCTGGCTTTGACAATGACATAGCACTGATTAAATTGAATAAC
AAAGTTGTAATCAATAGCAACATCACGCCTATTTGTCTGCCAAGAAAAGAAGCTGAATCC
TTTATGAGGACAGATGACATTGGAACTGCATCTGGATGGGGATTAACCCAAAGGGGTTTT
CTTGCTAGAAATCTAATGTATGTCGACATACCGATTGTTGACCATCAAAAATGTACTGCT
GCATATGAAAAGCCACCCTATCCAAGGGGAAGTGTAACTGCTAACATGCTTTGTGCTGGC
TTAGAAAGTGGGGGCAAGGACAGCTGCAGAGGTGACAGCGGAGGGGCACTGGTGTTTCTA
GATAGTGAAACAGAGAGGTGGTTTGTGGGAGGAATAGTGTCCTGGGGTTCCATGAATTGT
GGGGAAGCAGGTCAGTATGGAGTCTACACAAAAGTTATTAACTATATTCCCTGGATCGAG
AACATAATTAGTGATTTTTAA
Protein Properties
Number of Residues 686
Molecular Weight 75732.7
Theoretical pI 5.56
Pfam Domain Function
Signals
  • 1-15
Transmembrane Regions
  • None
Protein Sequence
>Mannan-binding lectin serine protease 2
MRLLTLLGLLCGSVATPLGPKWPEPVFGRLASPGFPGEYANDQERRWTLTAPPGYRLRLY
FTHFDLELSHLCEYDFVKLSSGAKVLATLCGQESTDTERAPGKDTFYSLGSSLDITFRSD
YSNEKPFTGFEAFYAAEDIDECQVAPGEAPTCDHHCHNHLGGFYCSCRAGYVLHRNKRTC
SALCSGQVFTQRSGELSSPEYPRPYPKLSSCTYSISLEEGFSVILDFVESFDVETHPETL
CPYDFLKIQTDREEHGPFCGKTLPHRIETKSNTVTITFVTDESGDHTGWKIHYTSTAHAC
PYPMAPPNGHVSPVQAKYILKDSFSIFCETGYELLQGHLPLKSFTAVCQKDGSWDRPMPA
CSIVDCGPPDYLPSGRVEYITGPGVTTYKAVIQYSCEETFYTMKVNDGKYVCEADGFWTS
SKGEKSLPVCEPVCGLSARTTGGRIYGGQKAKPGDFPWQVLILGGTTAAGALLYDNWVLT
AAHAVYEQKHDASALDIRMGTLKRLSPHYTQAWSEAVFIHEGYTHDAGFDNDIALIKLNN
KVVINSNITPICLPRKEAESFMRTDDIGTASGWGLTQRGFLARNLMYVDIPIVDHQKCTA
AYEKPPYPRGSVTANMLCAGLESGGKDSCRGDSGGALVFLDSETERWFVGGIVSWGSMNC
GEAGQYGVYTKVINYIPWIENIISDF
GenBank ID Protein 4007627
UniProtKB/Swiss-Prot ID O00187
UniProtKB/Swiss-Prot Entry Name MASP2_HUMAN
PDB IDs
GenBank Gene ID Y09926
GeneCard ID MASP2
GenAtlas ID MASP2
HGNC ID HGNC:6902
References
General References
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  3. Stover C, Endo Y, Takahashi M, Lynch NJ, Constantinescu C, Vorup-Jensen T, Thiel S, Friedl H, Hankeln T, Hall R, Gregory S, Fujita T, Schwaeble W: The human gene for mannan-binding lectin-associated serine protease-2 (MASP-2), the effector component of the lectin route of complement activation, is part of a tightly linked gene cluster on chromosome 1p36.2-3. Genes Immun. 2001 May;2(3):119-27. [PubMed:11426320 ]
  4. Thiel S, Vorup-Jensen T, Stover CM, Schwaeble W, Laursen SB, Poulsen K, Willis AC, Eggleton P, Hansen S, Holmskov U, Reid KB, Jensenius JC: A second serine protease associated with mannan-binding lectin that activates complement. Nature. 1997 Apr 3;386(6624):506-10. [PubMed:9087411 ]
  5. Takahashi M, Endo Y, Fujita T, Matsushita M: A truncated form of mannose-binding lectin-associated serine protease (MASP)-2 expressed by alternative polyadenylation is a component of the lectin complement pathway. Int Immunol. 1999 May;11(5):859-63. [PubMed:10330290 ]
  6. Stover CM, Thiel S, Thelen M, Lynch NJ, Vorup-Jensen T, Jensenius JC, Schwaeble WJ: Two constituents of the initiation complex of the mannan-binding lectin activation pathway of complement are encoded by a single structural gene. J Immunol. 1999 Mar 15;162(6):3481-90. [PubMed:10092804 ]
  7. Rasmussen HH, Orntoft TF, Wolf H, Celis JE: Towards a comprehensive database of proteins from the urine of patients with bladder cancer. J Urol. 1996 Jun;155(6):2113-9. [PubMed:8618346 ]
  8. Matsushita M, Thiel S, Jensenius JC, Terai I, Fujita T: Proteolytic activities of two types of mannose-binding lectin-associated serine protease. J Immunol. 2000 Sep 1;165(5):2637-42. [PubMed:10946292 ]
  9. Gregory LA, Thielens NM, Matsushita M, Sorensen R, Arlaud GJ, Fontecilla-Camps JC, Gaboriaud C: The X-ray structure of human mannan-binding lectin-associated protein 19 (MAp19) and its interaction site with mannan-binding lectin and L-ficolin. J Biol Chem. 2004 Jul 9;279(28):29391-7. Epub 2004 Apr 26. [PubMed:15117939 ]
  10. Harmat V, Gal P, Kardos J, Szilagyi K, Ambrus G, Vegh B, Naray-Szabo G, Zavodszky P: The structure of MBL-associated serine protease-2 reveals that identical substrate specificities of C1s and MASP-2 are realized through different sets of enzyme-substrate interactions. J Mol Biol. 2004 Oct 1;342(5):1533-46. [PubMed:15364579 ]
  11. Gal P, Harmat V, Kocsis A, Bian T, Barna L, Ambrus G, Vegh B, Balczer J, Sim RB, Naray-Szabo G, Zavodszky P: A true autoactivating enzyme. Structural insight into mannose-binding lectin-associated serine protease-2 activations. J Biol Chem. 2005 Sep 30;280(39):33435-44. Epub 2005 Jul 21. [PubMed:16040602 ]
  12. Stengaard-Pedersen K, Thiel S, Gadjeva M, Moller-Kristensen M, Sorensen R, Jensen LT, Sjoholm AG, Fugger L, Jensenius JC: Inherited deficiency of mannan-binding lectin-associated serine protease 2. N Engl J Med. 2003 Aug 7;349(6):554-60. [PubMed:12904520 ]
  13. Lozano F, Suarez B, Munoz A, Jensenius JC, Mensa J, Vives J, Horcajada JP: Novel MASP2 variants detected among North African and Sub-Saharan individuals. Tissue Antigens. 2005 Aug;66(2):131-5. [PubMed:16029433 ]