ID:MCFD2_HUMAN DESCRIPTION: RecName: Full=Multiple coagulation factor deficiency protein 2; AltName: Full=Neural stem cell-derived neuronal survival protein; Flags: Precursor; FUNCTION: The MCFD2-LMAN1 complex forms a specific cargo receptor for the ER-to-Golgi transport of selected proteins. Plays a role in the secretion of coagulation factors. SUBUNIT: Interacts in a calcium-dependent manner with LMAN1. SUBCELLULAR LOCATION: Endoplasmic reticulum-Golgi intermediate compartment. Endoplasmic reticulum. Golgi apparatus. DOMAIN: Essentially unstructured in the absence of calcium ions. Requires calcium ions for folding. DISEASE: Defects in MCFD2 are a cause of factor V and factor VIII combined deficiency type 2 (F5F8D2) [MIM:613625]; also known as multiple coagulation factor deficiency 2 (MCFD2). F5F8D2 is a blood coagulation disorder characterized by bleeding symptoms similar to those in hemophilia or parahemophilia, that are caused by single deficiency of FV or FVIII, respectively. The most common symptoms are epistaxis, menorrhagia, and excessive bleeding during or after trauma. Plasma levels of coagulation factors V and VIII are in the range of 5 to 30% of normal. SIMILARITY: Contains 2 EF-hand domains. SEQUENCE CAUTION: Sequence=CAD38756.1; Type=Erroneous initiation;
The RNAfold program from the Vienna RNA Package is used to perform the secondary structure predictions and folding calculations. The estimated folding energy is in kcal/mol. The more negative the energy, the more secondary structure the RNA is likely to have.
ModBase Predicted Comparative 3D Structure on Q8NI22
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Orthologous Genes in Other Species
Orthologies between human, mouse, and rat are computed by taking the best BLASTP hit, and filtering out non-syntenic hits. For more distant species reciprocal-best BLASTP hits are used. Note that the absence of an ortholog in the table below may reflect incomplete annotations in the other species rather than a true absence of the orthologous gene.
Biological Process: GO:0006888 ER to Golgi vesicle-mediated transport GO:0015031 protein transport GO:0016192 vesicle-mediated transport GO:0048208 COPII vesicle coating
BC037845 - Homo sapiens multiple coagulation factor deficiency 2, mRNA (cDNA clone MGC:42267 IMAGE:4815069), complete cds. BC040357 - Homo sapiens multiple coagulation factor deficiency 2, mRNA (cDNA clone MGC:9537 IMAGE:3925166), complete cds. AL833900 - Homo sapiens mRNA; cDNA DKFZp762M047 (from clone DKFZp762M047); complete cds. CR749562 - Homo sapiens mRNA; cDNA DKFZp686G21263 (from clone DKFZp686G21263). AK095006 - Homo sapiens cDNA FLJ37687 fis, clone BRHIP2014285. M23161 - Human transposon-like element mRNA. AF537214 - Homo sapiens multiple coagulation factor deficiency protein 2 (MCFD2) mRNA, complete cds. JD448997 - Sequence 430021 from Patent EP1572962. JD058475 - Sequence 39499 from Patent EP1572962. JD508376 - Sequence 489400 from Patent EP1572962. JD132939 - Sequence 113963 from Patent EP1572962. JD145821 - Sequence 126845 from Patent EP1572962. JD092628 - Sequence 73652 from Patent EP1572962. JD111854 - Sequence 92878 from Patent EP1572962. JD282938 - Sequence 263962 from Patent EP1572962. JD144387 - Sequence 125411 from Patent EP1572962. JD102895 - Sequence 83919 from Patent EP1572962. JD288738 - Sequence 269762 from Patent EP1572962. JD360707 - Sequence 341731 from Patent EP1572962. JD528700 - Sequence 509724 from Patent EP1572962. JD094602 - Sequence 75626 from Patent EP1572962. JD324854 - Sequence 305878 from Patent EP1572962. DQ577493 - Homo sapiens piRNA piR-45605, complete sequence. JD492203 - Sequence 473227 from Patent EP1572962. JD147044 - Sequence 128068 from Patent EP1572962. DQ570462 - Homo sapiens piRNA piR-30574, complete sequence. JD273866 - Sequence 254890 from Patent EP1572962. JD315596 - Sequence 296620 from Patent EP1572962. JD371975 - Sequence 352999 from Patent EP1572962. JD506482 - Sequence 487506 from Patent EP1572962. JD362595 - Sequence 343619 from Patent EP1572962. JD364089 - Sequence 345113 from Patent EP1572962. JD158272 - Sequence 139296 from Patent EP1572962. JD191771 - Sequence 172795 from Patent EP1572962. JD325676 - Sequence 306700 from Patent EP1572962. JD367122 - Sequence 348146 from Patent EP1572962. JD188859 - Sequence 169883 from Patent EP1572962. JD524382 - Sequence 505406 from Patent EP1572962. JD402044 - Sequence 383068 from Patent EP1572962. JD422401 - Sequence 403425 from Patent EP1572962. JD186471 - Sequence 167495 from Patent EP1572962. JD173667 - Sequence 154691 from Patent EP1572962. JD561940 - Sequence 542964 from Patent EP1572962. JD219228 - Sequence 200252 from Patent EP1572962. JD483274 - Sequence 464298 from Patent EP1572962. JD088981 - Sequence 70005 from Patent EP1572962. JD375211 - Sequence 356235 from Patent EP1572962. AK293889 - Homo sapiens cDNA FLJ59187 complete cds, highly similar to Multiple coagulation factor deficiency protein 2 homolog precursor. JD401080 - Sequence 382104 from Patent EP1572962. AK292127 - Homo sapiens cDNA FLJ75145 complete cds, highly similar to Homo sapiens multiple coagulation factor deficiency 2 (MCFD2),mRNA. JD512387 - Sequence 493411 from Patent EP1572962. JD291391 - Sequence 272415 from Patent EP1572962. JD327946 - Sequence 308970 from Patent EP1572962. JD224456 - Sequence 205480 from Patent EP1572962. AF475284 - Homo sapiens neural stem cell derived neuronal survival protein precursor (SDNSF) mRNA, complete cds. JD114164 - Sequence 95188 from Patent EP1572962. DQ891104 - Synthetic construct clone IMAGE:100003734; FLH169368.01X; RZPDo839B0696D multiple coagulation factor deficiency 2 (MCFD2) gene, encodes complete protein. DQ894285 - Synthetic construct Homo sapiens clone IMAGE:100008745; FLH169364.01L; RZPDo839B0695D multiple coagulation factor deficiency 2 (MCFD2) gene, encodes complete protein. CU691864 - Synthetic construct Homo sapiens gateway clone IMAGE:100017926 5' read MCFD2 mRNA. KJ895121 - Synthetic construct Homo sapiens clone ccsbBroadEn_04515 MCFD2 gene, encodes complete protein. KR709606 - Synthetic construct Homo sapiens clone CCSBHm_00003930 MCFD2 (MCFD2) mRNA, encodes complete protein. KR709607 - Synthetic construct Homo sapiens clone CCSBHm_00003931 MCFD2 (MCFD2) mRNA, encodes complete protein. KR709608 - Synthetic construct Homo sapiens clone CCSBHm_00003933 MCFD2 (MCFD2) mRNA, encodes complete protein. KR709609 - Synthetic construct Homo sapiens clone CCSBHm_00003934 MCFD2 (MCFD2) mRNA, encodes complete protein.
Biochemical and Signaling Pathways
Reactome (by CSHL, EBI, and GO)
Protein Q8NI22 (Reactome details) participates in the following event(s):
R-HSA-5694431 Hexameric LMAN1:MCFD2 bind glycosylated Factor V and VIII precursors R-HSA-947991 Transport of glycoproteins with Man8 (or Man9) N-glycans to the Golgi R-HSA-5694522 Inner coat assembly and cargo binding R-HSA-5694527 Loss of SAR1B GTPase R-HSA-5694446 BET1:GOSR2:STX5 bind v-SNARES on tethered vesicle R-HSA-5694441 CSNK1D phosphorylates SEC23 R-HSA-5694439 COPII coat binds TRAPPCII and RAB1:GDP R-HSA-203973 Vesicle budding R-HSA-5694417 SEC16 complex binds SAR1B:GTP:SEC23:SEC24 R-HSA-5694418 RAB1:GTP binds USO1 and GORASP1:GOLGA2 R-HSA-5694409 Nucleotide exchange on RAB1 R-HSA-204008 SEC31:SEC13 and v-SNARE recruitment R-HSA-5694530 Cargo concentration in the ER R-HSA-948021 Transport to the Golgi and subsequent modification R-HSA-199977 ER to Golgi Anterograde Transport R-HSA-446203 Asparagine N-linked glycosylation R-HSA-204005 COPII-mediated vesicle transport R-HSA-199991 Membrane Trafficking R-HSA-597592 Post-translational protein modification R-HSA-5653656 Vesicle-mediated transport R-HSA-392499 Metabolism of proteins