Progress on human acid-labile subunit deficiency
10.3760/cma.j.issn.1673-4408.2011.06.008
- VernacularTitle:人类酸不稳定亚单位缺乏症的研究进展
- Author:
Lina ZHANG
;
Liyang LIANG
- Publication Type:Journal Article
- Keywords:
Acid-labile subunit;
Insulin-like growth factor;
Insulin-like growth factor binding protein;
Short stature;
Insuline insensitivity
- From:
International Journal of Pediatrics
2011;38(6):551-553
- CountryChina
- Language:Chinese
-
Abstract:
Acid-lable subunit (ALS)plays an important role in stablizing the insulin-like growth factor ( IGF),which has an great impact on the process of human growth and develepment.Mutations of the human IGF ALS gene result in ALS deficiency.Some degree of insulin insensitivity,as well as high level of insulin,low serum concentrations of IGF- Ⅰ and IGF binding protein ( IGFBP)-3,short stature,growth retardation,was generally found in patients with human ALS deficiency.Meanwhile,bone mineral desity reduction only occures in some cases.However,the exact mechanisms of insulin insensitivity and bone mineral density reduction have still remained unclear.
