Hematopoietic effects of activating the Hedgehog pathway.

刺猬蛋白对人类的正常发育很重要。它们在大脑和发育中的肢体细胞上表达，并向邻近细胞提供重要信号，使大脑和肢体的发育能够正常发生。刺猬信号通路中的基因突变会导致先天性异常，如大脑无法正常折叠，四肢缩短或多出手指。刺猬蛋白还刺激成体干细胞的生长，成体干细胞负责维持许多成体组织，如皮肤，骨骼和毛发。然而，过度的刺猬信号可能会导致癌症，特别是脑部和皮肤癌症。刺猬蛋白刺激干细胞生长的能力提高了它们在干细胞扩增中的用途。这对脐带血干细胞特别有用，如果有足够的数量，可以用于治疗白血病患者。该项目将研究刺猬蛋白刺激造血干细胞的潜在用途。

The hedgehog proteins are important for normal human development. They are expressed on cells of the brain and developing limbs and provide important signals to neighbouring cells so that development of the brain and limbs can occur normally. Mutations in genes within the hedgehog signalling pathway lead to congenital abnormalities such as failure of the brain to fold properly and shortened limbs or extra digits. Hedgehog proteins also stimulate the growth of adult stem cells that are responsible for the maintenance of many adult tissues such as the skin, bone and hair. Excessive hedgehog signalling however can lead to cancers, particularly of the brain and skin. The ability of hedgehog proteins to stimulate the growth of stem cells raises their use for expansion of stem cells. This would be particularly useful for umbilical cord blood stem cells, which could be used to treat patients with leukemia if there were sufficient numbers. This project will examine the potential use of hedgehog proteins for stimulating blood stem cells.