FENIB

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Tang, P. FENIB. Encyclopedia. Available online: https://encyclopedia.pub/entry/6045 (accessed on 20 September 2026).

Tang P. FENIB. Encyclopedia. Available at: https://encyclopedia.pub/entry/6045. Accessed September 20, 2026.

Tang, Peter. "FENIB" Encyclopedia, https://encyclopedia.pub/entry/6045 (accessed September 20, 2026).

Tang, P. (2021, January 04). FENIB. In Encyclopedia. https://encyclopedia.pub/entry/6045

Tang, Peter. "FENIB." Encyclopedia. Web. 04 January, 2021.

FENIB

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This entry is adapted from the peer-reviewed paper https://medlineplus.gov/genetics/condition/familial-encephalopathy-with-neuroserpin-inclusion-bodies

Familial encephalopathy with neuroserpin inclusion bodies (FENIB) is a disorder that causes progressive dysfunction of the brain (encephalopathy).

genetic conditions

References

Bradshaw CB, Davis RL, Shrimpton AE, Holohan PD, Rea CB, Fieglin D, Kent P,Collins GH. Cognitive deficits associated with a recently reported familialneurodegenerative disease: familial encephalopathy with neuroserpin inclusionbodies. Arch Neurol. 2001 Sep;58(9):1429-34.
Coutelier M, Andries S, Ghariani S, Dan B, Duyckaerts C, van Rijckevorsel K,Raftopoulos C, Deconinck N, Sonderegger P, Scaravilli F, Vikkula M, Godfraind C. Neuroserpin mutation causes electrical status epilepticus of slow-wave sleep.Neurology. 2008 Jul 1;71(1):64-6. doi: 10.1212/01.wnl.0000316306.08751.28.
Davis RL, Holohan PD, Shrimpton AE, Tatum AH, Daucher J, Collins GH, Todd R,Bradshaw C, Kent P, Feiglin D, Rosenbaum A, Yerby MS, Shaw CM, Lacbawan F,Lawrence DA. Familial encephalopathy with neuroserpin inclusion bodies. Am JPathol. 1999 Dec;155(6):1901-13.
Davis RL, Shrimpton AE, Carrell RW, Lomas DA, Gerhard L, Baumann B, LawrenceDA, Yepes M, Kim TS, Ghetti B, Piccardo P, Takao M, Lacbawan F, Muenke M, Sifers RN, Bradshaw CB, Kent PF, Collins GH, Larocca D, Holohan PD. Association between conformational mutations in neuroserpin and onset and severity of dementia.Lancet. 2002 Jun 29;359(9325):2242-7. Erratum in: Lancet 2002 Oct5;360(9339):1102.
Davis RL, Shrimpton AE, Holohan PD, Bradshaw C, Feiglin D, Collins GH,Sonderegger P, Kinter J, Becker LM, Lacbawan F, Krasnewich D, Muenke M, Lawrence DA, Yerby MS, Shaw CM, Gooptu B, Elliott PR, Finch JT, Carrell RW, Lomas DA.Familial dementia caused by polymerization of mutant neuroserpin. Nature. 1999Sep 23;401(6751):376-9.
Galliciotti G, Sonderegger P. Neuroserpin. Front Biosci. 2006 Jan 1;11:33-45. Review.
Gourfinkel-An I, Duyckaerts C, Camuzat A, Meyrignac C, Sonderegger P, BaulacM, Brice A. Clinical and neuropathologic study of a French family with a mutationin the neuroserpin gene. Neurology. 2007 Jul 3;69(1):79-83.
Miranda E, MacLeod I, Davies MJ, Pérez J, Römisch K, Crowther DC, Lomas DA.The intracellular accumulation of polymeric neuroserpin explains the severity of the dementia FENIB. Hum Mol Genet. 2008 Jun 1;17(11):1527-39. doi:10.1093/hmg/ddn041.
Yepes M, Lawrence DA. Neuroserpin: a selective inhibitor of tissue-typeplasminogen activator in the central nervous system. Thromb Haemost. 2004Mar;91(3):457-64. Review.

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Subjects: Genetics & Heredity

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Update Date: 04 Jan 2021

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