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相似文献

1
Copper metabolism in mottled mouse mutants. The effect of copper therapy on lysyl oxidase activity in brindled (Mobr) mice.斑驳小鼠突变体中的铜代谢。铜疗法对花斑（Mobr）小鼠赖氨酰氧化酶活性的影响。
Biochem J. 1982 Feb 15;202(2):369-71. doi: 10.1042/bj2020369.
2
Copper metabolism in mottled mouse mutants: copper therapy of brindled (Mobr) mice.斑驳小鼠突变体中的铜代谢：对花斑（Mobr）小鼠的铜治疗。
Biochem J. 1979 Jun 15;180(3):605-12. doi: 10.1042/bj1800605.
3
Biochemical study on the critical period for treatment of the mottled brindled mouse.花斑小鼠治疗关键期的生化研究
J Neurochem. 1990 Sep;55(3):885-9. doi: 10.1111/j.1471-4159.1990.tb04574.x.
4
Reduced lysyl oxidase activity in skin fibroblasts from patients with Menkes' syndrome.门克斯综合征患者皮肤成纤维细胞中赖氨酰氧化酶活性降低。
Biochem J. 1980 Nov 15;192(2):579-86. doi: 10.1042/bj1920579.
5
Markedly reduced activity of lysyl oxidase in skin and aorta from a patient with Menkes' disease showing unusually severe connective tissue manifestations.患有门克斯病且表现出异常严重结缔组织症状的患者，其皮肤和主动脉中赖氨酰氧化酶的活性显著降低。
Pediatr Res. 1990 Aug;28(2):137-41. doi: 10.1203/00006450-199008000-00012.
6
Copper metabolism in mottled mouse (Mus musculus) mutants. Studies of blotchy (Moblo) mice and a comparison with brindled (Mobr) mice.斑驳小鼠（小家鼠）突变体中的铜代谢。斑点（Moblo）小鼠的研究以及与带条纹（Mobr）小鼠的比较。
Biochem J. 1981 Apr 15;196(1):81-8. doi: 10.1042/bj1960081.
7
Mutations in humans and animals which affect copper metabolism.影响铜代谢的人类和动物突变。
J Inherit Metab Dis. 1983;6 Suppl 1:44-50. doi: 10.1007/BF01811323.
8
Neurochemical and immunocytochemical studies of catecholamine system in the brindled mouse.
J Neuropathol Exp Neurol. 1991 Nov;50(6):793-808. doi: 10.1097/00005072-199111000-00010.
9
Expression and accumulation of lysyl oxidase, elastin, and type I procollagen in human Menkes and mottled mouse fibroblasts.
Arch Biochem Biophys. 1993 Mar;301(2):325-9. doi: 10.1006/abbi.1993.1151.
10
Lysyl oxidase.赖氨酰氧化酶
Int Rev Connect Tissue Res. 1979;8:73-118. doi: 10.1016/b978-0-12-363708-6.50009-6.

引用本文的文献

1
Mottled Mice and Non-Mammalian Models of Menkes Disease.斑驳小鼠与门克斯病的非哺乳动物模型
Front Mol Neurosci. 2015 Dec 18;8:72. doi: 10.3389/fnmol.2015.00072. eCollection 2015.
2
Investigation of iron metabolism in mice expressing a mutant Menke's copper transporting ATPase (Atp7a) protein with diminished activity (Brindled; Mo (Br) (/y) ).表达活性降低的 Menke's 铜转运 ATP 酶（Atp7a）突变蛋白的小鼠的铁代谢研究（斑纹；Mo（Br）（/y））。
PLoS One. 2013 Jun 11;8(6):e66010. doi: 10.1371/journal.pone.0066010. Print 2013.
3
ATP7A-related copper transport diseases-emerging concepts and future trends.ATP7A 相关铜转运疾病——新出现的概念和未来趋势。
Nat Rev Neurol. 2011 Jan;7(1):15-29. doi: 10.1038/nrneurol.2010.180.
4
Fibroblast silver loading for the diagnosis of Menkes disease.用于诊断门克斯病的成纤维细胞银染色法
J Med Genet. 1998 Oct;35(10):849-51. doi: 10.1136/jmg.35.10.849.
5
Type IX Ehlers-Danlos syndrome and Menkes syndrome: the decrease in lysyl oxidase activity is associated with a corresponding deficiency in the enzyme protein.IX型埃勒斯-当洛综合征和门克斯综合征：赖氨酰氧化酶活性降低与该酶蛋白相应缺乏有关。
Am J Hum Genet. 1985 Jul;37(4):798-808.
6
Similar splicing mutations of the Menkes/mottled copper-transporting ATPase gene in occipital horn syndrome and the blotchy mouse.枕角综合征和斑点小鼠中门克斯/斑驳铜转运ATP酶基因的类似剪接突变。
Am J Hum Genet. 1995 Mar;56(3):570-6.
7
Mutations in humans and animals which affect copper metabolism.影响铜代谢的人类和动物突变。
J Inherit Metab Dis. 1983;6 Suppl 1:44-50. doi: 10.1007/BF01811323.
8
Of mice and men, metals and mutations.关于小鼠与人、金属与突变。
J Med Genet. 1986 Apr;23(2):99-106. doi: 10.1136/jmg.23.2.99.
9
Comparisons of copper deficiency states in the murine mutants blotchy and brindled. Changes in copper-dependent enzyme activity in 13-day-old mice.小鼠突变体“斑点”和“有斑纹”中铜缺乏状态的比较。13日龄小鼠中铜依赖性酶活性的变化。
Biochem J. 1986 Aug 15;238(1):177-83. doi: 10.1042/bj2380177.
10
The relationship of excess copper accumulation by fibroblasts from the brindled mouse model of Menkes disease to the primary defect.来自门克斯病（Menkes disease）的斑驳小鼠模型的成纤维细胞中过量铜积累与原发性缺陷的关系。
Biochem J. 1990 Apr 15;267(2):417-22. doi: 10.1042/bj2670417.

本文引用的文献

1
Altered copper metabolism in cultured cells from human Menkes' syndrome and mottled mouse mutants.来自人类门克斯综合征和斑驳小鼠突变体的培养细胞中铜代谢的改变。
Biochem Genet. 1980 Feb;18(1-2):117-31. doi: 10.1007/BF00504364.
2
Copper metabolism in mottled mouse mutants. Defective placental transfer of 64Cu to foetal brindled (Mobr) mice.斑驳小鼠突变体中的铜代谢。64Cu向胎儿花斑（Mobr）小鼠的胎盘转运缺陷。
Biochem J. 1980 Feb 15;186(2):629-31. doi: 10.1042/bj1860629.
3
Copper metabolism of cultured fibroblasts from the brindled mouse (gene symbol Mobr).斑驳小鼠（基因符号Mobr）培养成纤维细胞的铜代谢
Proc Soc Exp Biol Med. 1981 Jan;166(1):153-6. doi: 10.3181/00379727-166-41038.
4
Reduced lysyl oxidase activity in skin fibroblasts from patients with Menkes' syndrome.门克斯综合征患者皮肤成纤维细胞中赖氨酰氧化酶活性降低。
Biochem J. 1980 Nov 15;192(2):579-86. doi: 10.1042/bj1920579.
5
Primary defect in copper transport underlies mottled mutants in the mouse.铜转运的原发性缺陷是小鼠斑驳突变体的基础。
Nature. 1974 Jun 28;249(460):852-4. doi: 10.1038/249852a0.
6
A sex-linked defect in the cross-linking of collagen and elastin associated with the mottled locus in mice.与小鼠斑驳位点相关的胶原蛋白和弹性蛋白交联中的性连锁缺陷。
J Exp Med. 1974 Jan 1;139(1):180-92. doi: 10.1084/jem.139.1.180.
7
Menkes disease: a biochemical abnormality in cultured human fibroblasts.门克斯病：培养的人成纤维细胞中的一种生化异常。
Proc Natl Acad Sci U S A. 1976 Feb;73(2):604-6. doi: 10.1073/pnas.73.2.604.
8
Menkes's syndrome. Report of a patient treated from 21 days of age with parenteral copper.门克斯综合征。一名自21日龄起接受胃肠外铜治疗的患者报告。
Arch Dis Child. 1978 Dec;53(12):956-8. doi: 10.1136/adc.53.12.956.
9
Copper metabolism in mottled mouse mutants: distribution of 64Cu in brindled (Mobr) mice.斑驳小鼠突变体中的铜代谢：64Cu在花斑（Mobr）小鼠中的分布。
Biochem J. 1979 Jun 15;180(3):613-9. doi: 10.1042/bj1800613.
10
Copper metabolism in mottled mouse mutants: copper therapy of brindled (Mobr) mice.斑驳小鼠突变体中的铜代谢：对花斑（Mobr）小鼠的铜治疗。
Biochem J. 1979 Jun 15;180(3):605-12. doi: 10.1042/bj1800605.

斑驳小鼠突变体中的铜代谢。铜疗法对花斑（Mobr）小鼠赖氨酰氧化酶活性的影响。

Copper metabolism in mottled mouse mutants. The effect of copper therapy on lysyl oxidase activity in brindled (Mobr) mice.

作者信息

Royce P M, Camakaris J, Mann J R, Danks D M

出版信息

Biochem J. 1982 Feb 15;202(2):369-71. doi: 10.1042/bj2020369.

DOI:10.1042/bj2020369
PMID:6124241
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1158120/
Abstract

Lysyl oxidase activity in extracts of skin from 1-day-old Mobr/Y mice was found to be between 50 and 60% of that in corresponding extracts from littermate +/Y mice of the same age. It was increased to 84-150% of that in the latter by prior treatment of the Mobr/Y mice at 7 days of age with a single subcutaneous injection of 50 micrograms of copper, retained as Cu+ in an alkyl polyether/sebacic acid solution. This suggests that in this form the copper is able to by-pass the block in copper metabolism and is deliverable to copper-requiring processes.

摘要

发现1日龄Mobr/Y小鼠皮肤提取物中的赖氨酰氧化酶活性为同年龄同窝+/Y小鼠相应提取物中酶活性的50%至60%。在7日龄时给Mobr/Y小鼠单次皮下注射50微克以Cu⁺形式保留在烷基聚醚/癸二酸溶液中的铜进行预处理后，其活性增加至后者的84%至150%。这表明以这种形式的铜能够绕过铜代谢中的障碍，并可输送至需要铜的过程。