音猬因子

音猬因子（英语：sonic hedgehog，简称SHH）是5种刺猬因子（hedgehog，简称HH，其他四种是沙漠刺猬因子（英语：DHH (hedgehog)），DHH、印度刺猬因子（英语：IHH (protein)），IHH、Echidna Hedgehog，EHH和Tiggywinkle Hedgehog，TwHH）中的一种。

音猬因子
3D structure of the signaling domain of the murine Sonic hedgehog from PDB: 1vhh​
有效结构 PDB 直系同源检索：PDBe, RCSB PDB查询代码列表 3HO5​, 3M1N​, 3MXW​
标识
代号	SHH; HHG1; HLP3; HPE3; MCOPCB5; SMMCI; TPT; TPTPS
扩展标识	遗传学：600725 鼠基因：98297 同源基因：30961 GeneCards: SHH Gene
基因本体论描述 分子功能 · glycoprotein binding · signal transducer activity · patched binding · calcium ion binding · protein binding · glycosaminoglycan binding · peptidase activity · zinc ion binding · laminin-1 binding 细胞成分 · extracellular region · extracellular space · extracellular space · membrane fraction · nucleus · endoplasmic reticulum · Golgi apparatus · plasma membrane · cell surface · transport vesicle · extracellular matrix · membrane raft 生物过程 · patterning of blood vessels · vasculogenesis · metanephros development · branching involved in ureteric bud morphogenesis · response to hypoxia · cell fate specification · neural crest cell migration · neural tube formation · vasculature development · heart looping · positive regulation of neuroblast proliferation · positive regulation of mesenchymal cell proliferation · osteoblast development · lymphoid progenitor cell differentiation · endocytosis · signal transduction · smoothened signaling pathway · positive regulation of hh target transcription factor activity · cell-cell signaling · determination of left/right symmetry · pattern specification process · ectoderm development · neuroblast proliferation · axon guidance · central nervous system development · ventral midline development · hindgut morphogenesis · digestive tract mesoderm development · heart development · blood coagulation · androgen metabolic process · cell death · positive regulation of cell proliferation · embryo development · anterior/posterior pattern formation · dorsal/ventral pattern formation · response to organic nitrogen · oligodendrocyte development · striated muscle tissue development · positive regulation of skeletal muscle cell proliferation · myotube differentiation · intein-mediated protein splicing · spinal cord dorsal/ventral patterning · ventral spinal cord interneuron specification · dorsal/ventral neural tube patterning · smoothened signaling pathway involved in regulation of cerebellar granule cell precursor cell proliferation · positive regulation of cerebellar granule cell precursor proliferation · telencephalon regionalization · establishment of cell polarity · regulation of proteolysis · positive regulation of Wnt receptor signaling pathway · lung development · embryonic limb morphogenesis · negative regulation of cell migration · male genitalia development · prostate gland development · regulation of epithelial cell differentiation · thyroid gland development · forebrain development · midbrain development · hindbrain development · pancreas development · hair follicle morphogenesis · response to estradiol stimulus · negative regulation of proteasomal ubiquitin-dependent protein catabolic process · response to retinoic acid · T cell differentiation in thymus · positive regulation of T cell differentiation in thymus · positive regulation of immature T cell proliferation in thymus · embryonic forelimb morphogenesis · embryonic hindlimb morphogenesis · regulation of cell proliferation · negative regulation of T cell proliferation · positive regulation of protein import into nucleus · odontogenesis of dentine-containing tooth · embryonic digit morphogenesis · camera-type eye development · negative regulation of apoptosis · CD4-positive or CD8-positive, alpha-beta T cell lineage commitment · tongue morphogenesis · skin development · positive thymic T cell selection · negative thymic T cell selection · intermediate filament organization · myoblast differentiation · response to ethanol · negative regulation of cell differentiation · positive regulation of transcription, DNA-dependent · positive regulation of transcription from RNA polymerase II promoter · positive regulation of photoreceptor cell differentiation · positive regulation of alpha-beta T cell differentiation · negative regulation of alpha-beta T cell differentiation · cell development · thymus development · embryonic digestive tract morphogenesis · embryonic organ development · developmental growth · embryonic foregut morphogenesis · positive regulation of skeletal muscle tissue development · organ formation · neuron fate commitment · response to axon injury · embryonic skeletal system development · positive regulation of oligodendrocyte differentiation · branching morphogenesis of a tube · male genitalia morphogenesis · inner ear development · formation of anatomical boundary · stem cell development · positive regulation of striated muscle cell differentiation · Bergmann glial cell differentiation · palate development · limb development · limb bud formation · positive regulation of penile erection · lung epithelium development · trachea morphogenesis · branching involved in salivary gland morphogenesis · bud outgrowth involved in lung branching · right lung development · left lung development · lung lobe morphogenesis · lung-associated mesenchyme development · primary prostatic bud elongation · prostate epithelial cord elongation · salivary gland cavitation · epithelial cell proliferation involved in salivary gland morphogenesis · regulation of prostatic bud formation · epithelial-mesenchymal signaling involved in prostate gland development · positive regulation of epithelial cell proliferation involved in prostate gland development · regulation of mesenchymal cell proliferation involved in prostate gland development · mesenchymal smoothened signaling pathway involved in prostate gland development · artery development · mesenchymal cell proliferation involved in lung development · sclerotome development · positive regulation of oligodendrocyte progenitor proliferation · cellular response to lithium ion · metanephric mesenchymal cell proliferation involved in metanephros development · regulation of mesenchymal cell proliferation involved in ureter development · multicellular structure septum development · negative regulation of canonical Wnt receptor signaling pathway · negative regulation of ureter smooth muscle cell differentiation · positive regulation of ureter smooth muscle cell differentiation · negative regulation of kidney smooth muscle cell differentiation · positive regulation of kidney smooth muscle cell differentiation Sources: Amigo / QuickGO
RNA表达模式
更多表达数据
直系同源体
物种	人类	小鼠
Entrez	6469	20423
Ensembl	ENSG00000164690	ENSMUSG00000002633
UniProt	Q15465	Q8C765
mRNA序列	NM_000193.2	NM_009170.3
蛋白序列	NP_000184.1	NP_033196.1
基因位置	Chr 7: 155.59 – 155.6 Mb	Chr 5: 28.78 – 28.79 Mb
PubMed查询	[1]	[2]
查 论 编

刺猬因子是重要的信号传导途径。而音猬因子则是刺猬信号传导途径中研究最透彻的配体，这种因子作为重要的形态发生素（Morphogen），在调节脊椎动物器官发育中起关键作用，比如它决定四肢以及脑脊髓正中线的形成。音猬因子在成年个体中也很重要，它控制成年体细胞的分裂。音猬因子的失控将导致癌症。

发表

这种刺猬基因(刺猬信号通路, hh)是由1995年诺贝尔生理学或医学奖得主艾瑞克·威斯乔斯以及克里斯汀·纽斯林-沃尔哈德所确认，他们的研究于1980年发表^[1]。这个发表更为他们带来了1995年的诺贝尔生理学或医学奖。

命名

当初于黑腹果蝇（Drosophila）身上进行研究时，发现当这类因子发生突变时，果蝇的幼虫表皮上会长满了短的倒刺，就好像刺猬的模样。因此就命名为“刺猬”（Hedgehog）。

及后当发现了更多此类型的基因后，为分别就以“刺猬”为基本去进行命名。当中有两种先被发现的刺猬因子 － 沙漠刺猬因子（英语：DHH (hedgehog)）（Desert Hedgehog，DHH）以及印度刺猬因子（英语：IHH (protein)）（Indian Hedgehog，IHH）就是以两种现实存在的刺猬去进行命名。而及后发现的“音猬因子”则是以世嘉一套相当有名的电玩主角刺猬索尼克（Sonic the Hedgehog，即“音速小子”或“超音鼠”）而进行命名。

研究

果蝇的刺猬因子在上世纪的90年代早期被克隆。除果蝇外，也有在其他非脊椎动物中克隆这类基因，如水蛭和海胆。

至于脊椎动物，其直系同源基因于1993年被克隆，包括的动物有小鼠，斑马鱼和鸡。之后不久，分别于1994年及1995年，第一个大鼠和人的刺猬因子也宣告克隆成功。

疾病

当音猬因子发生突变，会因腹侧正中线缺失而引起前脑发育畸形症Holoprosencephaly(HPE)。而音猬因子在成年个体中的失调亦会导致癌症。

逸闻

这种利用电玩去命名的原则也发生在前称“Pokemon”的Zbtb7因子（与《宠物小精灵》的名字相同），以及对视力相关的皮卡丘素（Pikachurin）（以宠物小精灵中的皮卡丘命名）。然前者却因专利与商标问题被迫更名。

另见

• 名称独特的化学物质列表