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61.努比亞山羊黏多醣症(G6S)基因致死效應之驗證

林德育 陳坤照 張溪泉 王炳煌 黃鈺嘉 吳明哲

行政院農業委員會畜產試驗所

山羊黏多醣症(G6S)為努比亞山羊之一重要遺傳缺陷，它是由於乙醯醣胺氨基硫酸酶基因單點核苷酸突變所導致的隱性遺傳之代謝異常疾病。為驗證此不良基因對努比亞山羊生產之影響，選用1頭帶有山羊黏多醣症基因雜合型的公羊與配12頭雜合型母羊，經配種繁殖後有8頭母羊受孕，共產出17頭仔羊，受孕率。所有仔羊經G6S基因型檢測結果，有6頭為正常型(TG)、7頭為雜合型(CG)，4頭為有病型(G6S)。有病型仔羊育生長至2月齡前即有75%(3/4)頭死亡，顯示山羊黏多醣症確實會造成仔羊生長早期死亡，而影響仔羊育成率。

關鍵語：努比亞山羊、山羊黏多醣症、致死效應。

AN EXPERIMENTAL INVESTIGATION ON lethal effect of CAPRINE MUCOPOLYSACCHARIDOSIS IIID Gene in Nubian GOAT

D. Y. Lin, K. J. Chen, H. C. Chang, P. H. Wang, Y. C. Huang and M. C. Wu

Livestock Research Institute, Council of Agriculture, Executive Yuan

Caprine Mucopolysaccharidosis IIID is an important inherited deficiency in Nubian goat.  It was caused by a point mutation of N-acetylglucosamine 6-sulfatase(G6S) gene.  To verified the caprine mucopolysaccharidosis IIID gene effect on surviving rate of Nubian kids, one heterozygote buck of G6S were mated with 12 heterozygote nanny goats in mating season. Eight nannies were pregnant and produced seventeen kids.  The pregnant rate and kidding rate were 67%(8/12) and 213%(17/8), respectively.  All of these kids were genotyped for G6S.  Six kids are normal (TG, test free of G6S), 7 kids are heterozygote (GC, G6S carrier) and 4 kids are affected (G6S).  Three of the four affected kids died within two months after birth. Results showed that caprine mucopolysaccharidosis IIID could cause kid died at early stage, and affected the rearing rate of kids.

Key Words: Nubian goat, Caprine Mucopolysaccharidosis, Lethal effect