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söndag 29 juli 2018

SOST geeni ja sen tuote sklerostiini. Uutta tietoa vuodelta 2017

SOST gene (17q21.31), Sclerostin

sclerostin precursor [Homo sapiens]

NCBI Reference Sequence: NP_079513.1


LOCUS       NP_079513                213 aa            linear   PRI 23-JUN-2018
DEFINITION  sclerostin precursor [Homo sapiens].
ACCESSION   NP_079513
VERSION     NP_079513.1
DBSOURCE    REFSEQ: accession NM_025237.2
KEYWORDS    RefSeq.
SOURCE      Homo sapiens (human)
  ORGANISM  Homo sapiens
            Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi;
            Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini;
            Catarrhini; Hominidae; Homo.
REFERENCE   1  (residues 1 to 213)
  AUTHORS   Wu T, Wang LN, Tang DR and Sun FY.
  TITLE     SOST silencing promotes proliferation and invasion and reduces
            apoptosis of retinoblastoma cells by activating Wnt/beta-catenin
            signaling pathway
  JOURNAL   Gene Ther. 24 (7), 399-407 (2017)
   PUBMED   28485721
  REMARK    GeneRIF: SOST silencing promotes the proliferation, invasion and
            migration, and decreases the apoptosis of human retinoblastoma
            cells by activating the Wnt/beta-catenin signaling pathway.
REFERENCE   2  (residues 1 to 213)
  AUTHORS   Tartaglione L, Pasquali M, Rotondi S, Muci ML, Leonangeli C,
            Farcomeni A, Fassino V and Mazzaferro S.
  TITLE     Interactions of sclerostin with FGF23, soluble klotho and vitamin D
            in renal transplantation
  JOURNAL   PLoS ONE 12 (5), e0178637 (2017)
   PUBMED   28558021
  REMARK    GeneRIF: Sclerostin levels in KTR are normal and influenced more by
            bone turnover than by eGFR. Its involvement with other hormones of
            mineral homeostasis (FGF23/Klotho and Vitamin D) is part of the
            sophisticated cross-talk between bone and the kidney
            Publication Status: Online-Only
REFERENCE   3  (residues 1 to 213)
  AUTHORS   Behets GJ, Viaene L, Meijers B, Blocki F, Brandenburg VM, Verhulst
            A, D'Haese PC and Evenepoel P.
  TITLE     Circulating levels of sclerostin but not DKK1 associate with
            laboratory parameters of CKD-MBD
  JOURNAL   PLoS ONE 12 (5), e0176411 (2017)
   PUBMED   28493902
  REMARK    GeneRIF: In chronic kidney disease, serum levels of the Wnt
            inhibitors DKK1 and sclerostin are unrelated, indicating different
            sites of origin and/ or different regulatory mechanisms.
            Sclerostin, as opposed to DKK1, may qualify as a biomarker of
            chronic kidney disease-mineral and bone and mineral disorder
            (CKD-MBD), particularly in dialysis patients.
            Publication Status: Online-Only
REFERENCE   4  (residues 1 to 213)
  AUTHORS   Saritekin I, Acikgoz S, Bayraktaroglu T, Kuzu F, Can M, Guven B,
            Mungan G, Buyukuysal C and Sarikaya S.
  TITLE     Sclerostin and bone metabolism markers in hyperthyroidism before
            treatment and interrelations between them
  JOURNAL   Acta Biochim. Pol. 64 (4), 597-602 (2017)
   PUBMED   29059259
  REMARK    GeneRIF: No difference was found in the serum sclerostin levels
            between the hyperthyroidism patients and healthy control.
REFERENCE   5  (residues 1 to 213)
  AUTHORS   Shi J, Ying H, Du J and Shen B.
  TITLE     Serum Sclerostin Levels in Patients with Ankylosing Spondylitis and
            Rheumatoid Arthritis: A Systematic Review and Meta-Analysis
  JOURNAL   Biomed Res Int 2017, 9295313 (2017)
   PUBMED   28553652
  REMARK    GeneRIF: The difference of serum sclerostin levels in Ankylosing
            Spondylitis and Rheumatoid Arthritis patients was not significantly
            different from HC, indicating that the sclerostin may not associate
            with the development of Ankylosing Spondylitis and Rheumatoid
            Arthritis.
            Review article
            Erratum:[Biomed Res Int. 2017;2017:3953474. PMID: 29181394]
REFERENCE   6  (residues 1 to 213)
  AUTHORS   Staehling-Hampton K, Proll S, Paeper BW, Zhao L, Charmley P, Brown
            A, Gardner JC, Galas D, Schatzman RC, Beighton P, Papapoulos S,
            Hamersma H and Brunkow ME.
  TITLE     A 52-kb deletion in the SOST-MEOX1 intergenic region on 17q12-q21
            is associated with van Buchem disease in the Dutch population
  JOURNAL   Am. J. Med. Genet. 110 (2), 144-152 (2002)
   PUBMED   12116252
REFERENCE   7  (residues 1 to 213)
  AUTHORS   Balemans W, Patel N, Ebeling M, Van Hul E, Wuyts W, Lacza C,
            Dioszegi M, Dikkers FG, Hildering P, Willems PJ, Verheij JB,
            Lindpaintner K, Vickery B, Foernzler D and Van Hul W.
  TITLE     Identification of a 52 kb deletion downstream of the SOST gene in
            patients with van Buchem disease
  JOURNAL   J. Med. Genet. 39 (2), 91-97 (2002)
   PUBMED   11836356
  REMARK    GeneRIF: A 52 kb deletion has been identified downstream of the
            SOST gene in patients with van Buchem disease.
REFERENCE   8  (residues 1 to 213)
  AUTHORS   Balemans W, Ebeling M, Patel N, Van Hul E, Olson P, Dioszegi M,
            Lacza C, Wuyts W, Van Den Ende J, Willems P, Paes-Alves AF, Hill S,
            Bueno M, Ramos FJ, Tacconi P, Dikkers FG, Stratakis C, Lindpaintner
            K, Vickery B, Foernzler D and Van Hul W.
  TITLE     Increased bone density in sclerosteosis is due to the deficiency of
            a novel secreted protein (SOST)
  JOURNAL   Hum. Mol. Genet. 10 (5), 537-543 (2001)
   PUBMED   11181578
REFERENCE   9  (residues 1 to 213)
  AUTHORS   Brunkow ME, Gardner JC, Van Ness J, Paeper BW, Kovacevich BR, Proll
            S, Skonier JE, Zhao L, Sabo PJ, Fu Y, Alisch RS, Gillett L, Colbert
            T, Tacconi P, Galas D, Hamersma H, Beighton P and Mulligan J.
  TITLE     Bone dysplasia sclerosteosis results from loss of the SOST gene
            product, a novel cystine knot-containing protein
  JOURNAL   Am. J. Hum. Genet. 68 (3), 577-589 (2001)
   PUBMED   11179006
REFERENCE   10 (residues 1 to 213)
  AUTHORS   Beighton,P.H., Hamersma,H. and Brunkow,M.E.
  TITLE     SOST-Related Sclerosing Bone Dysplasias
  JOURNAL   (in) Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens
            K and Amemiya A (Eds.);
            GENEREVIEWS((R));
            (1993)
   PUBMED   20301406
COMMENT     REVIEWED REFSEQ: This record has been curated by NCBI staff. The
            reference sequence was derived from AF326739.1.
            This sequence is a reference standard in the RefSeqGene project.
            
            Summary: Sclerostin is a secreted glycoprotein with a C-terminal
            cysteine knot-like (CTCK) domain and sequence similarity to the DAN
            (differential screening-selected gene aberrative in neuroblastoma)
            family of bone morphogenetic protein (BMP) antagonists.
            Loss-of-function mutations in this gene are associated with an
            autosomal-recessive disorder, sclerosteosis, which causes
            progressive bone overgrowth. A deletion downstream of this gene,
            which causes reduced sclerostin expression, is associated with a
            milder form of the disorder called van Buchem disease. [provided by
            RefSeq, Jul 2008].
            
            Publication Note:  This RefSeq record includes a subset of the
            publications that are available for this gene. Please see the Gene
            record to access additional publications.
            
            ##Evidence-Data-START##
            Transcript exon combination :: AF326739.1, AY358627.1 [ECO:0000332]
            RNAseq introns              :: single sample supports all introns
                                           SAMEA1970526, SAMEA2142363
                                           [ECO:0000348]
            ##Evidence-Data-END##
FEATURES             Location/Qualifiers
     source          1..213
                     /organism="Homo sapiens"
                     /db_xref="taxon:9606"
                     /chromosome="17"
                     /map="17q21.31"
     Protein         1..213
                     /product="sclerostin precursor"
                     /calculated_mol_wt=21522
     sig_peptide     1..23
                     /inference="COORDINATES: ab initio prediction:SignalP:4.0"
                     /calculated_mol_wt=2526
     mat_peptide     24..213
                     /product="sclerostin"
                     /calculated_mol_wt=21522
     Region          28..210
                     /region_name="GHB_like"
                     /note="Glycoprotein hormone beta chain homologues;
                     cl21545"
                     /db_xref="CDD:328788"
     CDS             1..213
                     /gene="SOST"
                     /gene_synonym="CDD; DAND6; SOST1; VBCH"
                     /coded_by="NM_025237.2:48..689"
                     /db_xref="CCDS:CCDS11468.1"
                     /db_xref="GeneID:50964"
                     /db_xref="HGNC:HGNC:13771"
                     /db_xref="MIM:605740"
ORIGIN      
        1 mqlplalclv cllvhtafrv vegqgwqafk ndateiipel geypepppel ennktmnrae
       61 nggrpphhpf etkdvseysc relhftryvt dgpcrsakpv telvcsgqcg parllpnaig
      121 rgkwwrpsgp dfrcipdryr aqrvqllcpg geaprarkvr lvasckckrl trfhnqselk
      181 dfgteaarpq kgrkprprar sakanqaele nay
//

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