Human Pancreatic Secretory Trypsin Inhibitor

Marchbank, Tania; Freeman, Tom C.

doi:10.1159/000007485

Cited by 51 publications

(12 citation statements)

References 27 publications

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“…3d, h, l, p, and t). These Wndings were consistent with the previously reported data of Marchbank et al (1998). Without exception, the X-gal staining data from 16.5 dpc up to 0.5 dpp showed the similar expression pattern of Spink3 within the pancreas.…”

Section: Spink3 Expression In the Pancreassupporting

confidence: 93%

Expression pattern of serine protease inhibitor kazal type 3 (Spink3) during mouse embryonic development

Wang

Ohmuraya

Hirota

et al. 2008

Histochem Cell Biol

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Recent evidence shows that the serine protease inhibitor Kazal type 3 (Spink3) has more diverse functions than expected. To gain insight into its function, we analyzed the spatiotemporal expression profile of Spink3, using in situ hybridization (ISH) and a Spink3+/lacZ knock-in mouse, in which lacZ was inserted into the Spink3 locus. Spink3lacZ expression was first observed in the foregut, midgut, hindgut and the forebrain/midbrain junction region at 9.5 days post coitus (dpc). In the pancreas, Spink3 mRNA was detected at 11.5 dpc, before formation of the typical shape of the exocrine structure of the pancreas. Acinar cell expression was clearly identified by 13.5 dpc. After differentiation of the intestinal tract, Spink3lacZ expression was observed in the large intestine at 11.5 dpc, followed by expression in the small intestine at 13.5 dpc, before appearance of intestinal digestive enzymes. Spink3 mRNA and Spink3lacZ activity were also detected in other tissues, including the mesonephric tubules and the urogenital ridge at 11.5 dpc, the genital swelling at 13.5 dpc, the ductus epididymis at 17.5 dpc, and the seminal vesicle at 8 weeks. These data suggest that Spink3 may play important roles in proliferation and/or differentiation of various cell types during development.

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Section: Spink3 Expression In the Pancreassupporting

confidence: 93%

Expression pattern of serine protease inhibitor kazal type 3 (Spink3) during mouse embryonic development

Wang

Ohmuraya

Hirota

et al. 2008

Histochem Cell Biol

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“…Given the relationship between serine protease activity and apoptosis, serine protease inhibitors such as SPINKs deserve special attention. SPINK1, SPINK3, and SPINK5 have been found to have trypsin-inhibitory activities (13,22,30). Dysregulation of SPINK proteins is known to cause severe diseases.…”

Section: Discussionmentioning

confidence: 99%

“…The first member of the SPINK family, human SPINK1 (mouse Spink3), also known as pancreatic secretory trypsin inhibitor was discovered by Kazal (12). Previous studies and our in silico searches indicate that at least 13 SPINK family members are expressed in diverse tissues (13)(14)(15)(16)(17)(18). SPINK2, the only SPINK family member expressed in testes, was first identified in humans (11,19); however, informa-tion on mouse Spink2 has not yet been reported.…”

mentioning

confidence: 86%

Impaired Spermatogenesis and Fertility in Mice Carrying a Mutation in the Spink2 Gene Expressed Predominantly in Testes

Lee

Park

Jin

et al. 2011

Journal of Biological Chemistry

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Spermatogenesis is a complex process involving an intrinsic genetic program composed of germ cell-specific and -predominant genes. In this study, we investigated the mouse Spink2 (serine protease inhibitor Kazal-type 2) gene, which belongs to the SPINK family of proteins characterized by the presence of a Kazal-type serine protease inhibitor-pancreatic secretory trypsin inhibitor domain. We showed that recombinant mouse SPINK2 has trypsin-inhibitory activity. Distribution analyses revealed that Spink2 is transcribed strongly in the testis and weakly in the epididymis, but is not detected in other mouse tissues. Expression of Spink2 is specific to germ cells in the testis and is first evident at the pachytene spermatocyte stage. Immunoblot analyses demonstrated that SPINK2 protein is present in male germ cells at all developmental stages, including in testicular spermatogenic cells, testicular sperm, and mature sperm. To elucidate the functional role of SPINK2 in vivo, we generated mutant mice with diminished levels of SPINK2 using a gene trap mutagenesis approach. Mutant male mice exhibit significantly impaired fertility; further phenotypic analyses revealed that testicular integrity is disrupted, resulting in a reduction in sperm number. Moreover, we found that testes from mutant mice exhibit abnormal spermatogenesis and germ cell apoptosis accompanied by elevated serine protease activity. Our studies thus provide the first demonstration that SPINK2 is required for maintaining normal spermatogenesis and potentially regulates serine protease-mediated apoptosis in male germ cells.

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“…This substitution does not seem to affect splicing of SPINK1 mRNA, as assessed by nested RT-PCR from a rectal biopsy of the patient (not shown). However, it leads to a missense mutation R65Q at an amino acid position that is conserved in rat and mouse, although some variability exists in cattle 11. Interestingly, the same patient had also been found to be heterozygous for a nonsense mutation of the CFTR gene, Y1092X in exon 17b8(table 1).…”

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confidence: 99%

Low prevalence of SPINK1 gene mutations in adult patients with chronic idiopathic pancreatitis

Ockenga

2001

Journal of Medical Genetics

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(SMN2) generates alternatively spliced variants lacking the C-terminal sequence.5 7 The SMN region contains low copy repeats triggering homologous recombination events. Indeed, approximately 95% of SMA patients lack both SMN1 genes owing to either deletion or gene conversion. 4 In SMA patients who lack only one SMN1 gene, allelic intragenic mutations have been identified, confirming the involvement of SMN1 in the pathogenesis of SMA.

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Human Pancreatic Secretory Trypsin Inhibitor

Cited by 51 publications

References 27 publications

Expression pattern of serine protease inhibitor kazal type 3 (Spink3) during mouse embryonic development

Expression pattern of serine protease inhibitor kazal type 3 (Spink3) during mouse embryonic development

Impaired Spermatogenesis and Fertility in Mice Carrying a Mutation in the Spink2 Gene Expressed Predominantly in Testes

Low prevalence of SPINK1 gene mutations in adult patients with chronic idiopathic pancreatitis

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