KEGG   VARIANT: 50814v1
Entry
50814v1                      Variant                               
Name
NSDHL deficiency
Type
Loss of function
Gene
NSDHL  NAD(P) dependent 3-beta-hydroxysteroid dehydrogenase NSDHL [KO:K07748]
Organism
hsa_var Human gene variants (Homo sapiens)
Variation
mutations
OmimVar: 300275
Network
nt06034  Cholesterol biosynthesis
Disease
H00496  Congenital hemidysplasia with ichthyosiform nevus and limb defects (CHILD)
H01917  CK syndrome
Reference
  Authors
Konig A, Happle R, Bornholdt D, Engel H, Grzeschik KH
  Title
Mutations in the NSDHL gene, encoding a 3beta-hydroxysteroid dehydrogenase, cause CHILD syndrome.
  Journal
Reference
  Authors
McLarren KW, Severson TM, du Souich C, Stockton DW, Kratz LE, Cunningham D, Hendson G, Morin RD, Wu D, Paul JE, An J, Nelson TN, Chou A, DeBarber AE, Merkens LS, Michaud JL, Waters PJ, Yin J, McGillivray B, Demos M, Rouleau GA, Grzeschik KH, Smith R, Tarpey PS, Shears D, Schwartz CE, Gecz J, Stratton MR, Arbour L, Hurlburt J, Van Allen MI, Herman GE, Zhao Y, Moore R, Kelley RI, Jones SJ, Steiner RD, Raymond FL, Marra MA, Boerkoel CF
  Title
Hypomorphic temperature-sensitive alleles of NSDHL cause CK syndrome.
  Journal
Am J Hum Genet 87:905-14 (2010)
DOI:10.1016/j.ajhg.2010.11.004
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