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Sterol-sensing domain

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56: 55:(HMGCR), involved in the biosynthesis of cholesterol. This was the first protein with an SSD to be discovered. Upon binding to cholesterol, this protein undergoes 48:
have shown that these proteins can be grouped in seven different families according to their SSDs. The following SSD-containing proteins represent each family:
44:
Sterol-sensing domains are present in various proteins involved in key aspects of cholesterol homeostasis and signalling. Multiple sequence alignments using
66: 133: 191: 62: 137: 72: 108: 296: 120: 111:
and also cytokinesis. Its SSD binds the cholesterol moiety of hedgehog, and it is essential for its activity.
247:
Pasca di Magliano, M; Hebrok, M (December 2003). "Hedgehog signalling in cancer formation and maintenance".
117:(DISP1, DISP2, DISP3), also involved in hedgehog signaling. DISP is required for the secretion of hedgehog. 65:(SCAP), which regulates transcription of genes with sterol response elements by proteolytically activating 114: 212:
Kuwabara, PE; Labouesse, M (April 2002). "The sterol-sensing domain: multiple families, a unique role?".
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groups. This type of domain is present in proteins involved in cholesterol metabolism and signalling.
141: 29: 272: 264: 229: 52: 256: 221: 149: 145: 25: 225: 290: 276: 181: 88: 123:(PTR), structurally similar to patched. Its functions are not fully understood. 268: 233: 186: 161: 96: 45: 69:(SREBPs). This was the second SSD-containing protein to be discovered. 84: 33: 260: 104: 100: 140:. Mutations in patched are associated with a variety of cancers ( 78: 75:(7DHCR), involved in the last step of cholesterol biosynthesis. 59:. The SSD is not required for the catalytic activity of HMGCR. 81:(NPC1), involved in intracellular cholesterol transport. 57:
endoplasmic-reticulum-associated protein degradation
136:(SLOS). Mutations in NPC1 have been shown to cause 87:(NPC1L1), a regulator of cholesterol absorption in 28:which consists of 180 amino acids forming five 8: 67:sterol regulatory element-binding proteins 173: 7: 14: 192:European Bioinformatics Institute 132:Mutations in 7DHCR are linked to 63:SREBP cleavage-activating protein 73:7-dehydrocholesterol reductase 1: 226:10.1016/s0168-9525(02)02640-9 138:Niemann–Pick disease, type C 85:Niemann-Pick type C1-like 1 313: 134:Smith–Lemli–Opitz syndrome 109:hedgehog signaling pathway 182:"Sterol-sensing domain" 121:Patched-related protein 249:Nature Reviews. Cancer 30:transmembrane segments 18:sterol-sensing domain 142:basal cell carcinoma 79:Niemann-Pick type C1 107:), involved in the 32:capable of binding 214:Trends in Genetics 53:HMG-CoA reductase 304: 281: 280: 244: 238: 237: 209: 203: 202: 200: 198: 178: 150:rhabdomyosarcoma 312: 311: 307: 306: 305: 303: 302: 301: 297:Protein domains 287: 286: 285: 284: 261:10.1038/nrc1229 246: 245: 241: 211: 210: 206: 196: 194: 180: 179: 175: 170: 158: 146:medulloblastoma 130: 42: 12: 11: 5: 310: 308: 300: 299: 289: 288: 283: 282: 255:(12): 903–11. 239: 220:(4): 193–201. 204: 172: 171: 169: 166: 165: 164: 157: 154: 129: 126: 125: 124: 118: 112: 94: 93: 92: 76: 70: 60: 41: 38: 26:protein domain 13: 10: 9: 6: 4: 3: 2: 309: 298: 295: 294: 292: 278: 274: 270: 266: 262: 258: 254: 250: 243: 240: 235: 231: 227: 223: 219: 215: 208: 205: 193: 189: 188: 183: 177: 174: 167: 163: 160: 159: 155: 153: 151: 147: 143: 139: 135: 127: 122: 119: 116: 113: 110: 106: 102: 98: 95: 90: 86: 83: 82: 80: 77: 74: 71: 68: 64: 61: 58: 54: 51: 50: 49: 47: 39: 37: 35: 31: 27: 23: 19: 252: 248: 242: 217: 213: 207: 195:. Retrieved 185: 176: 131: 43: 21: 17: 15: 89:enterocytes 168:References 115:Dispatched 46:Clustal W 291:Category 277:34050826 269:14737121 234:11932020 197:25 March 187:InterPro 156:See also 40:Function 162:INSIG-1 128:Disease 97:Patched 24:) is a 275:  267:  232:  34:sterol 273:S2CID 105:PTCH2 101:PTCH1 265:PMID 230:PMID 199:2015 257:doi 222:doi 152:). 22:SSD 293:: 271:. 263:. 251:. 228:. 218:18 216:. 190:. 184:. 148:, 144:, 103:, 16:A 279:. 259:: 253:3 236:. 224:: 201:. 99:( 91:. 20:(

Index

protein domain
transmembrane segments
sterol
Clustal W
HMG-CoA reductase
endoplasmic-reticulum-associated protein degradation
SREBP cleavage-activating protein
sterol regulatory element-binding proteins
7-dehydrocholesterol reductase
Niemann-Pick type C1
Niemann-Pick type C1-like 1
enterocytes
Patched
PTCH1
PTCH2
hedgehog signaling pathway
Dispatched
Patched-related protein
Smith–Lemli–Opitz syndrome
Niemann–Pick disease, type C
basal cell carcinoma
medulloblastoma
rhabdomyosarcoma
INSIG-1
"Sterol-sensing domain"
InterPro
European Bioinformatics Institute
doi
10.1016/s0168-9525(02)02640-9
PMID

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