Characterization and chromosomal localization of ELANH2, the gene encoding human monocyte/neutrophil elastase inhibitor.
Human monocyte/neutrophil elastase inhibitor (HEI) is a protease inhibitor of the serpin superfamily that rapidly inactivates neutrophil elastase, proteinase-3, and possibly cathepsin-G in vitro and, by regulating these potent proteases, is thought to prevent tissue damage at inflammatory sites. The HEI gene (ELANH2) was characterized by amplifying intron regions using cDNA-specific primers. Intron positions of ELANH2 were found to be homologous to intron positions in the genes for the serpin molecules chicken ovalbumin and human plasminogen activator inhibitor-2 (PLANH2). Because serpin superfamily genes in general have widely different organizational patterns, the shared organization of these genes strengthens the evidence that they form a subgroup or family, the "ovalbumin-related serpin" ("Ov-serpin") family. By amplifying DNA of a somatic cell hybrid panel, ELANH2 was unambiguously localized to chromosome 6. The use of a panel of radiation and somatic cell hybrids specific for chromosome 6 refined the localization of ELANH2 to the short arm telomeric of D6S89, F13A, and D6S202 at 6p24-pter. Another Ov-serpin gene PI6 (placental thrombin inhibitor) was colocalized to the same region, thus defining an Ov-serpin locus on chromosome 6 in addition to the previously defined PLANH2-containing Ov-serpin locus on chromosome 18.
Center for Blood Research, Harvard Medical School, Boston, Massachusetts 02115, USA.
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E
Evans
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J
Cooley
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Remold-O'Donnell
D001483:Base Sequence; D002874:Chromosome Mapping; D002896:Chromosomes, Human, Pair 6; D005796:Genes; D006801:Humans; D006822:Hybrid Cells; D007438:Introns / Q000235:genetics; D019272:Leukocyte Elastase; D008969:Molecular Sequence Data; D005810:Multigene Family; D010196:Pancreatic Elastase / Q000037:antagonists & inhibitors*; D011506:Proteins / Q000235:genetics*; D015843:Serpins / Q000235:genetics
D011506:Proteins; D015843:Serpins; C075177:SERPINB1 protein, human; D010196:Pancreatic Elastase; D019272:Leukocyte Elastase