Owen C A, McCall J T
Am J Vet Res. 1983 Apr;44(4):694-6.
Copper (Cu) was assayed in serial liver biopsy specimens of 5 pups resulting from a mating of 2 Bedlington Terrier carriers of inherited Cu toxicosis; the latter were sibling offspring of an affected Bedlington Terrier and a normal dog. Between 5 and 7 months of age, 1 of the pups had acceptable hepatic Cu values in each of 6 specimens. The hepatic concentration of Cu in another pup increased steadily from 801 to 3,874 micrograms/g dry weight. The other 3 pups may be heterozygotes (carriers); in 1--the hepatic Cu peaked at 1,043 micrograms/g at 9 months, in the 2nd--at 636 micrograms/g at 7 months, and in the 3rd--at 492 micrograms/g at 7 months. Acceptable concentrations were present in these 3 dogs at 9 to 14 months. Results in the present study indicate that heterozygotes may have 1 normal and 1 abnormal controller gene for regulating their hepatic Cu metabolism, thus accounting for the increased hepatic Cu concentration only in the early months of life. The affected Bedlington Terrier with 2 abnormal genes was unable to suppress the progressive hepatic accumulation of Cu. Paired liver biopsy specimens obtained at 5 to 7 months and at 14 or 15 months of are might distinguish the heterozygote from the normal and the affected Bedlington Terrier pups.
对2只遗传性铜中毒贝德灵顿梗携带者交配所生的5只幼犬的系列肝脏活检标本进行了铜（Cu）含量测定；这2只携带者是一只患病贝德灵顿梗和一只正常犬的同胞后代。在5至7月龄期间，1只幼犬的6份标本中每份的肝脏铜含量均在可接受范围内。另一只幼犬肝脏中的铜浓度从801微克/克干重稳步升至3874微克/克干重。另外3只幼犬可能是杂合子（携带者）；其中1只——肝脏铜含量在9月龄时达到峰值1043微克/克，第2只——在7月龄时为636微克/克，第3只——在7月龄时为492微克/克。这3只犬在9至14月龄时铜含量处于可接受浓度。本研究结果表明，杂合子可能有1个正常和1个异常的调控基因来调节其肝脏铜代谢，因此仅在生命早期肝脏铜浓度会升高。具有2个异常基因的患病贝德灵顿梗无法抑制肝脏中铜的渐进性蓄积。在5至7月龄以及14或15月龄时获取的配对肝脏活检标本可能会区分杂合子幼犬与正常和患病的贝德灵顿梗幼犬。
