中文摘要：

目的探究胰腺酶原基因在混合型卟啉症模型斑马鱼中的表达特征。方法以原卟啉原氧化酶缺陷的混合型卟啉症斑马鱼和野生型斑马鱼为材料，利用胰腺酶原基因RNA探针进行原位杂交，从而确定胰腺酶原基因在突变体和野生型斑马鱼中的表达差异。结果胰腺酶原基因以及胰腺转录因子ptfla在混合型卟啉症斑马鱼中表达水平明显下调，氯高铁血红素处理后可以明显上调胰腺酶原基因以及胰腺转录因子ptfla在突变体中的表达水平。结论血红素调控胰腺酶原基因在混合型卟啉症斑马鱼中的表达。

英文摘要：

Objective To investigate the expression patterns of pancreatic zymogen genes in the variegate porphyria model zebrafish.Methods The embryos and larvae of protoporphyrinogen oxidase-deficient montalcino zebrafish and wild-type zebrafish were collected and probes of six pancreatic zymogen genes were made to determine differential expressions of these genes in the mutant and wild-type zebrafish through in situ hybridization.Results The expression levels of pancreatic zymogen genes and pancreatic transcription factor la were downregulated in the variegate porphyria model zebrafish.Hemin treatment can rescue the expression of these six pancreatic zymogen genes and ptfl a in montalcino larvae.Conclusion Heme regulates the expression of pancreatic zymogen genes and pancreatic transcription factor la in the zebrafish variegate porphyria model.