# 音猬因子[编辑]

| 音猬因子 |
| --- |
| 3D structure of the signaling domain of the murine Sonic hedgehog from PDB: 1vhh​ |
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| 有效结构 |
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| PDB | 直系同源检索：PDBe, RCSB
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| PDB查询代码列表 |
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| 3HO5​, 3M1N​, 3MXW​

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| 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 |
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| 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 |

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| RNA表达模式 |
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| 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
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**音蝟因子**（英語：sonic hedgehog，简称**SHH**）是5種刺蝟因子（hedgehog，简称**HH**，其他四种是沙漠刺猬因子，**DHH**、印度刺猬因子，**IHH**、Echidna Hedgehog，**EHH**和Tiggywinkle Hedgehog，**TwHH**）中的一種。

刺蝟因子是重要的信号传导途径。而音蝟因子則是刺蝟信号传导途径中研究最透彻的配体，這種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腦脊髓正中線的形成。音蝟因子在成年个体中也很重要，它控制成年体细胞的分裂。音蝟因子的失控将导致癌症。

## 發表

這種刺猬基因(刺猬信号通路, *hh*)是由1995年諾貝爾生理學或醫學獎得主艾瑞克·威斯喬斯以及克里斯汀·紐斯林-沃爾哈德所確認，他們的研究於1980年發表。這個發表更為他們帶來了1995年的諾貝爾生理學或醫學獎。

## 命名

當初於黑腹果蠅（*Drosophila*）身上進行研究時，發現當這類因子發生突變時，果蠅的幼蟲表皮上會長滿了短的倒刺，就好像刺蝟的模樣。因此就命名為「刺蝟」（Hedgehog）。

及後當發現了更多此類型的基因後，為分別就以「刺蝟」為基本去進行命名。當中有兩種先被發現的刺蝟因子 － 沙漠刺猬因子（Desert Hedgehog，DHH）以及印度刺猬因子（Indian Hedgehog，IHH）就是以兩種現實存在的刺蝟去進行命名。而及後發現的「音蝟因子」則是以世嘉一套相當有名的電玩主角刺蝟索尼克（Sonic the Hedgehog，即「音速小子」或「超音鼠」）而進行命名。

## 研究

果蝇的刺蝟因子在上世纪的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這類基因，如水蛭和海胆。

至於脊椎动物，其直系同源基因于1993年被克隆，包括的動物有小鼠，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蝟因子也宣告克隆成功。

## 疾病

當音蝟因子發生突變，會因腹側正中線缺失而引起前腦發育畸形症Holoprosencephaly(HPE)。而音蝟因子在成年个体中的失调亦会导致癌症。

## 逸聞

這種利用電玩去命名的原則也發生在前稱「Pokemon」的Zbtb7因子（與《寵物小精靈》的名字相同），以及對視力相關的皮卡丘素（Pikachurin）（以寵物小精靈中的皮卡丘命名）。然前者卻因專利與商標問題被迫更名。

## 另見

* 名稱獨特的化學物質列表

## 參考文獻