protein

Recombinant Human Neutrophil elastase

MBS957715

0.05 mg (E-Coli)

180 USD

Recombinant Protein

Recombinant Human Neutrophil elastase

Neutrophil elastase

Bone marrow serine protease; Elastase-2; Human leukocyte elastase; HLE: Medullasin; PMN elastase

neutrophil elastase preproprotein; Neutrophil elastase; neutrophil elastase; elastase, neutrophil expressed; Bone marrow serine protease; Elastase-2; Human leukocyte elastase; HLE; Medullasin; PMN elastase

ELANE

ELANE; ELANE; GE; NE; HLE; HNE; ELA2; SCN1; PMN-E; ELA2; HLE

ELNE_HUMAN

E Coli or Yeast or Baculovirus or Mammalian Cell

30-267; Mature full length protein

267

IVGGRRARPHAWPFMVSLQLRGGHFCGATLIAPNFVMSA
AHCVANVNVRAVRVVLGAHNLSRREPTRQVFAVQRIFEN
GYDPVNLLNDIVILQLNGSATINANVQVAQLPAQGRRLG
NGVQCLAMGWGLLGRNRGIASVLQELNVTVVTSLCRRSN
VCTLVRGRQAGVCFGDSGSPLVCNGLIHGIASFVRGGCA
SGLYPDAFAPVAQFVNWIDSIIQRSEDNPCPHPRDPDPA
SRTH

Greater than 90% as determined by SDS-PAGE.

Liquid containing glycerol; lyophilization may be available upon request.

Store at -20 degree C, for extended storage, conserve at -20 degree C or -80 degree C.

Signal Transduction

Modifies the functions of natural killer cells, monocytes and granulocytes. Inhibits C5a-dependent neutrophil enzyme release and chemotaxis.

Nucleotide sequence of human bone marrow serine protease (medullasin) gene.Nakamura H., Okano K., Aoki Y., Shimizu H., Naruto M.Nucleic Acids Res. 15:9601-9601(1987) Structure of the human neutrophil elastase gene.Takahashi H., Nukiwa T., Yoshimura K., Quick C.D., States D.J., Holmes M.D., Whang-Peng J., Knutsen T., Crystal R.G.J. Biol. Chem. 263:14739-14747(1988) The human neutrophil elastase gene. Analysis of the nucleotide sequence reveals three distinct classes of repetitive DNA.Farley D., Travis J., Salvesen G.Biol. Chem. Hoppe-Seyler 370:737-744(1989) Functional expression of human leukocyte elastase (HLE) /medullasin in eukaryotic cells.Okano K., Aoki Y., Shimizu H., Naruto M.Biochem. Biophys. Res. Commun. 167:1326-1332(1990) NIEHS SNPs program Molecular cloning of complementary DNA for human medullasin an inflammatory serine protease in bone marrow cells.Okano K., Aoki Y., Sakurai T., Kajitani M., Kanai S., Shimazu T., Shimizu H., Naruto M.J. Biochem. 102:13-16(1987) Primary structure of human neutrophil elastase.Sinha S., Watorek W., Karr S., Giles J., Bode W., Travis J.Proc. Natl. Acad. Sci. U.S.A. 84:2228-2232(1987) Travis J., Giles P.J., Porcelli L., Reilly C.F., Baugh R., Powers J.(In) Protein degradation in health and disease, Ciba Foundation Symposium, pp.75:51-68, Excerpta Medica, Amsterdam and Oxford (1980) Antibiotic proteins of human polymorphonuclear leukocytes.Gabay J.E., Scott R.W., Campanelli D., Griffith J., Wilde C., Marra M.N., Seeger M., Nathan C.F.Proc. Natl. Acad. Sci. U.S.A. 86:5610-5614(1989) Use of proteinase 3 purified by reverse phase HPLC to detect autoantibodies in systemic vasculitis.Gaskin G., Kendal H., Coulthart A., Turner N., Pusey C.D.J. Immunol. Methods 180:25-33(1995) Myelomonocytic cell lineage expression of the neutrophil elastase gene.Takahashi H., Nukiwa T., Basset P., Cystal R.G.J. Biol. Chem. 263:2543-2547(1988) Molecular cloning of human neutrophil elastase.Farley D., Salvesen G.S., Travis J.Biol. Chem. Hoppe-Seyler 369:3-7(1988) The primary structure and elastinolytic activity of medullasin (a serine protease of bone marrow) .Aoki Y., Hase T.Biochem. Biophys. Res. Commun. 178:501-506(1991) Human leukocyte elastase and cathepsin G are specific inhibitors of C5a-dependent neutrophil enzyme release and chemotaxis.Tralau T., Meyer-Hoffert U., Schroder J.M., Wiedow O.Exp. Dermatol. 13:316-325(2004) A novel notch protein, N2N, targeted by neutrophil elastase and implicated in hereditary neutropenia.Duan Z., Li F.-Q., Wechsler J., Meade-White K., Williams K., Benson K.F., Horwitz M.Mol. Cell. Biol. 24:58-70(2004) Glycoproteomics analysis of human liver tissue by combination of multiple enzyme digestion and hydrazide chemistry.Chen R., Jiang X., Sun D., Han G., Wang F., Ye M., Wang L., Zou H.J. Proteome Res. 8:651-661(2009) Initial characterization of the human central proteome.Burkard T.R., Planyavsky M., Kaupe I., Breitwieser F.P., Buerckstuemmer T., Bennett K.L., Superti-Furga G., Colinge J.BMC Syst. Biol. 5:17-17(2011) Structure of human neutrophil elastase in complex with a peptide chloromethyl ketone inhibitor at 1.84-A resolution.Navia M.A., McKeever B.M., Springer J.P., Lin T.-Y., Williams H.R., Fluder E.M., Dorn C.P., Hoogsteen K.Proc. Natl. Acad. Sci. U.S.A. 86:7-11(1989) The refined 2.3-A crystal structure of human leukocyte elastase in a complex with a valine chloromethyl ketone inhibitor.Wei A.-Z., Mayr I., Bode W.FEBS Lett. 234:367-373(1988) X-ray crystal structure of the complex of human leukocyte elastase (PMN elastase) and the third domain of the turkey ovomucoid inhibitor.Bode W., Wei A.-Z., Huber R., Meyer E., Travis J., Neumann S.EMBO J. 5:2453-2458(1986) Mutations in ELA2, encoding neutrophil elastase, define a 21-day biological clock in cyclic haematopoiesis.Horwitz M., Benson K.F., Person R.E., Aprikyan A.G., Dale D.C.Nat. Genet. 23:433-436(1999) Mutations in the gene encoding neutrophil elastase in congenital and cyclic neutropenia.Dale D.C., Person R.E., Bolyard A.A., Aprikyan A.G., Bos C., Bonilla M.A., Boxer L.A., Kannourakis G., Zeidler C., Welte K., Benson K.F., Horwitz M.Blood 96:2317-2322(2000) Mutations in the ELA2 gene encoding neutrophil elastase are present in most patients with sporadic severe congenital neutropenia but only in some patients with the familial form of the disease.Ancliff P.J., Gale R.E., Liesner R., Hann I.M., Linch D.C.Blood 98:2645-2650(2001) Paternal mosaicism proves the pathogenic nature of mutations in neutrophil elastase in severe congenital neutropenia.Ancliff P.J., Gale R.E., Watts M.J., Liesner R., Hann I.M., Strobel S., Linch D.C.Blood 100:707-709(2002) Mutations in the ELA2 gene correlate with more severe expression of neutropenia a study of 81 patients from the French Neutropenia Register.Bellanne-Chantelot C., Clauin S., Leblanc T., Cassinat B., Rodrigues-Lima F., Beaufils S., Vaury C., Barkaoui M., Fenneteau O., Maier-Redelsperger M., Chomienne C., Donadieu J.Blood 103:4119-4125(2004) Double de novo mutations of ELA2 in cyclic and severe congenital neutropenia.Salipante S.J., Benson K.F., Luty J., Hadavi V., Kariminejad R., Kariminejad M.H., Rezaei N., Horwitz M.S.Hum. Mutat. 28:874-881(2007) A novel mutation Ala57Val of the ELA2 gene in a Korean boy with severe congenital neutropenia.Lee S.T., Yoon H.S., Kim H.J., Lee J.H., Park J.H., Kim S.H., Seo J.J., Im H.J.Ann. Hematol. 88:593-595(2009) Homozygous HAX1 mutations in severe congenital neutropenia patients with sporadic disease a novel mutation in two unrelated British kindreds.Smith B.N., Ancliff P.J., Pizzey A., Khwaja A., Linch D.C., Gale R.E.Br. J. Haematol. 144:762-770(2009) Ela2 mutations and clinical manifestations in familial congenital neutropenia.Shiohara M., Shigemura T., Saito S., Tanaka M., Yanagisawa R., Sakashita K., Asada H., Ishii E., Koike K., Chin M., Kobayashi M., Koike K.J. Pediatr. Hematol. Oncol. 31:319-324(2009) Digenic mutations in severe congenital neutropenia.Germeshausen M., Zeidler C., Stuhrmann M., Lanciotti M., Ballmaier M., Welte K.Haematologica 95:1207-1210(2010) Pegfilgrastim in children with severe congenital neutropenia.Fioredda F., Calvillo M., Lanciotti M., Lanza T., Giunti L., Castagnola E., Lorenzi I., Tonelli R., Ghezzi P., Dufour C.Pediatr. Blood Cancer 54:465-467(2010) Severe congenital neutropenia in a multigenerational family with a novel neutrophil elastase (ELANE) mutation.van de Vosse E., Verhard E.M., Tool A.J., de Visser A.W., Kuijpers T.W., Hiemstra P.S., van Dissel J.T.Ann. Hematol. 90:151-158(2011) Four novel ELANE mutations in patients with congenital neutropenia.Kurnikova M., Maschan M., Dinova E., Shagina I., Finogenova N., Mamedova E., Polovtseva T., Shagin D., Shcherbina A.Pediatr. Blood Cancer 57:332-335(2011) The spectrum of ELANE mutations and their implications in severe congenital and cyclic neutropenia.Germeshausen M., Deerberg S., Peter Y., Reimer C., Kratz C.P., Ballmaier M.Hum. Mutat. 34:905-914(2013)

4503549

NP_001963.1

NM_001972.3

P08246

52.9kD

Activation Of Matrix Metalloproteinases Pathway (1270258); C-MYB Transcription Factor Network Pathway (138073); Collagen Degradation Pathway (1270259); Degradation Of The Extracellular Matrix Pathway (1270257); Extracellular Matrix Organization Pathway (1270244); Systemic Lupus Erythematosus Pathway (83122); Systemic Lupus Erythematosus Pathway (534); Transcriptional Misregulation In Cancer Pathway (523016); Transcriptional Misregulation In Cancer Pathway (522987); Amb2 Integrin Signaling Pathway (137945)

Elastases form a subfamily of serine proteases that hydrolyze many proteins in addition to elastin. Humans have six elastase genes which encode structurally similar proteins. The encoded preproprotein is proteolytically processed to generate the active protease. Following activation, this protease hydrolyzes proteins within specialized neutrophil lysosomes, called azurophil granules, as well as proteins of the extracellular matrix. The enzyme may play a role in degenerative and inflammatory diseases through proteolysis of collagen-IV and elastin. This protein also degrades the outer membrane protein A (OmpA) of E. coli as well as the virulence factors of such bacteria as Shigella, Salmonella and Yersinia. Mutations in this gene are associated with cyclic neutropenia and severe congenital neutropenia (SCN). This gene is present in a gene cluster on chromosome 19. [provided by RefSeq, Jan 2016]

ELANE: Modifies the functions of natural killer cells, monocytes and granulocytes. Inhibits C5a-dependent neutrophil enzyme release and chemotaxis. Defects in ELANE are a cause of cyclic haematopoiesis (CH); also known as cyclic neutropenia. CH is an autosomal dominant disease in which blood-cell production from the bone marrow oscillates with 21-day periodicity. Circulating neutrophils vary between almost normal numbers and zero. During intervals of neutropenia, affected individuals are at risk for opportunistic infection. Monocytes, platelets, lymphocytes and reticulocytes also cycle with the same frequency. Defects in ELANE are the cause of neutropenia severe congenital autosomal dominant type 1 (SCN1). SCN1 is a disorder of hematopoiesis characterized by a maturation arrest of granulopoiesis at the level of promyelocytes with peripheral blood absolute neutrophil counts below 0.5 x 10(9)/l and early onset of severe bacterial infections. Belongs to the peptidase S1 family. Elastase subfamily. Protein type: Cell cycle regulation; Protease; Motility/polarity/chemotaxis; EC 3.4.21.37; Cell surface. Chromosomal Location of Human Ortholog: 19p13.3. Cellular Component: cell surface; cytoplasm; extracellular region; extracellular space; secretory granule; transcriptional repressor complex. Molecular Function: cytokine binding; endopeptidase activity; heparin binding; peptidase activity; protease binding; protein binding; serine-type endopeptidase activity. Biological Process: acute inflammatory response to antigenic stimulus; cellular calcium ion homeostasis; collagen catabolic process; defense response to bacterium; extracellular matrix disassembly; extracellular matrix organization and biogenesis; leukocyte migration; negative regulation of chemokine biosynthetic process; negative regulation of chemotaxis; negative regulation of inflammatory response; negative regulation of interleukin-8 biosynthetic process; negative regulation of transcription from RNA polymerase II promoter; phagocytosis; positive regulation of immune response; positive regulation of interleukin-8 biosynthetic process; positive regulation of MAP kinase activity; positive regulation of smooth muscle cell proliferation; protein catabolic process; proteolysis; response to lipopolysaccharide; response to UV; response to yeast. Disease: Cyclic Neutropenia; Neutropenia, Severe Congenital, 1, Autosomal Dominant

0.05 mg (E-Coli)

180 USD

0.05 mg (Yeast)

260

0.2 mg (E-Coli)

490

0.5 mg (E-Coli)

715

0.2 mg (Yeast)

835