Disease mechanisms in hereditary sensory and autonomic neuropathies

Inherited peripheral neuropathies are common monogenically inherited diseases of the peripheral nervous system. In the most common variant, i.e., the hereditary motor and sensory neuropathies, both motor and sensory nerves are affected. In contrast, sensory abnormalities predominate or are exclusive...
Ausführliche Beschreibung

Gespeichert in:

Autor*in:	Nathalie Verpoorten [verfasserIn] Peter De Jonghe [verfasserIn] Vincent Timmerman [verfasserIn]

Format:	E-Artikel
Sprache:	Englisch

Erschienen:	2006

Schlagwörter:	Hereditary sensory and autonomic neuropathies Molecular genetics Disease mechanisms

Übergeordnetes Werk:	In: Neurobiology of Disease - Elsevier, 2021, 21(2006), 2, Seite 247-255
Übergeordnetes Werk:	volume:21 ; year:2006 ; number:2 ; pages:247-255

Links:	Link aufrufen Link aufrufen Link aufrufen Journal toc

DOI / URN:	10.1016/j.nbd.2005.08.004

Katalog-ID:	DOAJ069114382

Internformat


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allfields	10.1016/j.nbd.2005.08.004 doi (DE-627)DOAJ069114382 (DE-599)DOAJ4935c730e0084e87ae4b855c20b9ecea DE-627 ger DE-627 rakwb eng RC321-571 Nathalie Verpoorten verfasserin aut Disease mechanisms in hereditary sensory and autonomic neuropathies 2006 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Inherited peripheral neuropathies are common monogenically inherited diseases of the peripheral nervous system. In the most common variant, i.e., the hereditary motor and sensory neuropathies, both motor and sensory nerves are affected. In contrast, sensory abnormalities predominate or are exclusively present in hereditary sensory and autonomic neuropathies (HSAN). HSAN are clinically and genetically heterogeneous and are subdivided according to mode of inheritance, age of onset and clinical evolution. In recent years, 6 disease-causing genes have been identified for autosomal dominant and recessive HSAN. However, vesicular transport and axonal trafficking seem important common pathways leading to degeneration of sensory and autonomic neurons. This review discusses the HSAN-related genes and their biological role in the disease mechanisms leading to HSAN. Hereditary sensory and autonomic neuropathies Molecular genetics Disease mechanisms Neurosciences. Biological psychiatry. Neuropsychiatry Peter De Jonghe verfasserin aut Vincent Timmerman verfasserin aut In Neurobiology of Disease Elsevier, 2021 21(2006), 2, Seite 247-255 (DE-627)268125414 (DE-600)1471408-5 1095953X nnns volume:21 year:2006 number:2 pages:247-255 https://doi.org/10.1016/j.nbd.2005.08.004 kostenfrei https://doaj.org/article/4935c730e0084e87ae4b855c20b9ecea kostenfrei http://www.sciencedirect.com/science/article/pii/S0969996105002305 kostenfrei https://doaj.org/toc/1095-953X Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_165 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_370 GBV_ILN_602 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2010 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2034 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2106 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2122 GBV_ILN_2143 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2470 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4125 GBV_ILN_4242 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4326 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4338 GBV_ILN_4700 AR 21 2006 2 247-255
spelling	10.1016/j.nbd.2005.08.004 doi (DE-627)DOAJ069114382 (DE-599)DOAJ4935c730e0084e87ae4b855c20b9ecea DE-627 ger DE-627 rakwb eng RC321-571 Nathalie Verpoorten verfasserin aut Disease mechanisms in hereditary sensory and autonomic neuropathies 2006 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Inherited peripheral neuropathies are common monogenically inherited diseases of the peripheral nervous system. In the most common variant, i.e., the hereditary motor and sensory neuropathies, both motor and sensory nerves are affected. In contrast, sensory abnormalities predominate or are exclusively present in hereditary sensory and autonomic neuropathies (HSAN). HSAN are clinically and genetically heterogeneous and are subdivided according to mode of inheritance, age of onset and clinical evolution. In recent years, 6 disease-causing genes have been identified for autosomal dominant and recessive HSAN. However, vesicular transport and axonal trafficking seem important common pathways leading to degeneration of sensory and autonomic neurons. This review discusses the HSAN-related genes and their biological role in the disease mechanisms leading to HSAN. Hereditary sensory and autonomic neuropathies Molecular genetics Disease mechanisms Neurosciences. Biological psychiatry. Neuropsychiatry Peter De Jonghe verfasserin aut Vincent Timmerman verfasserin aut In Neurobiology of Disease Elsevier, 2021 21(2006), 2, Seite 247-255 (DE-627)268125414 (DE-600)1471408-5 1095953X nnns volume:21 year:2006 number:2 pages:247-255 https://doi.org/10.1016/j.nbd.2005.08.004 kostenfrei https://doaj.org/article/4935c730e0084e87ae4b855c20b9ecea kostenfrei http://www.sciencedirect.com/science/article/pii/S0969996105002305 kostenfrei https://doaj.org/toc/1095-953X Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_165 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_370 GBV_ILN_602 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2010 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2034 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2106 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2122 GBV_ILN_2143 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2470 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4125 GBV_ILN_4242 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4326 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4338 GBV_ILN_4700 AR 21 2006 2 247-255
allfields_unstemmed	10.1016/j.nbd.2005.08.004 doi (DE-627)DOAJ069114382 (DE-599)DOAJ4935c730e0084e87ae4b855c20b9ecea DE-627 ger DE-627 rakwb eng RC321-571 Nathalie Verpoorten verfasserin aut Disease mechanisms in hereditary sensory and autonomic neuropathies 2006 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Inherited peripheral neuropathies are common monogenically inherited diseases of the peripheral nervous system. In the most common variant, i.e., the hereditary motor and sensory neuropathies, both motor and sensory nerves are affected. In contrast, sensory abnormalities predominate or are exclusively present in hereditary sensory and autonomic neuropathies (HSAN). HSAN are clinically and genetically heterogeneous and are subdivided according to mode of inheritance, age of onset and clinical evolution. In recent years, 6 disease-causing genes have been identified for autosomal dominant and recessive HSAN. However, vesicular transport and axonal trafficking seem important common pathways leading to degeneration of sensory and autonomic neurons. This review discusses the HSAN-related genes and their biological role in the disease mechanisms leading to HSAN. Hereditary sensory and autonomic neuropathies Molecular genetics Disease mechanisms Neurosciences. Biological psychiatry. Neuropsychiatry Peter De Jonghe verfasserin aut Vincent Timmerman verfasserin aut In Neurobiology of Disease Elsevier, 2021 21(2006), 2, Seite 247-255 (DE-627)268125414 (DE-600)1471408-5 1095953X nnns volume:21 year:2006 number:2 pages:247-255 https://doi.org/10.1016/j.nbd.2005.08.004 kostenfrei https://doaj.org/article/4935c730e0084e87ae4b855c20b9ecea kostenfrei http://www.sciencedirect.com/science/article/pii/S0969996105002305 kostenfrei https://doaj.org/toc/1095-953X Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_165 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_370 GBV_ILN_602 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2010 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2034 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2106 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2122 GBV_ILN_2143 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2470 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4125 GBV_ILN_4242 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4326 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4338 GBV_ILN_4700 AR 21 2006 2 247-255
allfieldsGer	10.1016/j.nbd.2005.08.004 doi (DE-627)DOAJ069114382 (DE-599)DOAJ4935c730e0084e87ae4b855c20b9ecea DE-627 ger DE-627 rakwb eng RC321-571 Nathalie Verpoorten verfasserin aut Disease mechanisms in hereditary sensory and autonomic neuropathies 2006 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Inherited peripheral neuropathies are common monogenically inherited diseases of the peripheral nervous system. In the most common variant, i.e., the hereditary motor and sensory neuropathies, both motor and sensory nerves are affected. In contrast, sensory abnormalities predominate or are exclusively present in hereditary sensory and autonomic neuropathies (HSAN). HSAN are clinically and genetically heterogeneous and are subdivided according to mode of inheritance, age of onset and clinical evolution. In recent years, 6 disease-causing genes have been identified for autosomal dominant and recessive HSAN. However, vesicular transport and axonal trafficking seem important common pathways leading to degeneration of sensory and autonomic neurons. This review discusses the HSAN-related genes and their biological role in the disease mechanisms leading to HSAN. Hereditary sensory and autonomic neuropathies Molecular genetics Disease mechanisms Neurosciences. Biological psychiatry. Neuropsychiatry Peter De Jonghe verfasserin aut Vincent Timmerman verfasserin aut In Neurobiology of Disease Elsevier, 2021 21(2006), 2, Seite 247-255 (DE-627)268125414 (DE-600)1471408-5 1095953X nnns volume:21 year:2006 number:2 pages:247-255 https://doi.org/10.1016/j.nbd.2005.08.004 kostenfrei https://doaj.org/article/4935c730e0084e87ae4b855c20b9ecea kostenfrei http://www.sciencedirect.com/science/article/pii/S0969996105002305 kostenfrei https://doaj.org/toc/1095-953X Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_165 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_370 GBV_ILN_602 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2010 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2034 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2106 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2122 GBV_ILN_2143 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2470 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4125 GBV_ILN_4242 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4326 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4338 GBV_ILN_4700 AR 21 2006 2 247-255
allfieldsSound	10.1016/j.nbd.2005.08.004 doi (DE-627)DOAJ069114382 (DE-599)DOAJ4935c730e0084e87ae4b855c20b9ecea DE-627 ger DE-627 rakwb eng RC321-571 Nathalie Verpoorten verfasserin aut Disease mechanisms in hereditary sensory and autonomic neuropathies 2006 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Inherited peripheral neuropathies are common monogenically inherited diseases of the peripheral nervous system. In the most common variant, i.e., the hereditary motor and sensory neuropathies, both motor and sensory nerves are affected. In contrast, sensory abnormalities predominate or are exclusively present in hereditary sensory and autonomic neuropathies (HSAN). HSAN are clinically and genetically heterogeneous and are subdivided according to mode of inheritance, age of onset and clinical evolution. In recent years, 6 disease-causing genes have been identified for autosomal dominant and recessive HSAN. However, vesicular transport and axonal trafficking seem important common pathways leading to degeneration of sensory and autonomic neurons. This review discusses the HSAN-related genes and their biological role in the disease mechanisms leading to HSAN. Hereditary sensory and autonomic neuropathies Molecular genetics Disease mechanisms Neurosciences. Biological psychiatry. Neuropsychiatry Peter De Jonghe verfasserin aut Vincent Timmerman verfasserin aut In Neurobiology of Disease Elsevier, 2021 21(2006), 2, Seite 247-255 (DE-627)268125414 (DE-600)1471408-5 1095953X nnns volume:21 year:2006 number:2 pages:247-255 https://doi.org/10.1016/j.nbd.2005.08.004 kostenfrei https://doaj.org/article/4935c730e0084e87ae4b855c20b9ecea kostenfrei http://www.sciencedirect.com/science/article/pii/S0969996105002305 kostenfrei https://doaj.org/toc/1095-953X Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_165 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_370 GBV_ILN_602 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2010 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2034 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2106 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2122 GBV_ILN_2143 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2470 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4125 GBV_ILN_4242 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4326 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4338 GBV_ILN_4700 AR 21 2006 2 247-255
language	English
source	In Neurobiology of Disease 21(2006), 2, Seite 247-255 volume:21 year:2006 number:2 pages:247-255
sourceStr	In Neurobiology of Disease 21(2006), 2, Seite 247-255 volume:21 year:2006 number:2 pages:247-255
format_phy_str_mv	Article
institution	findex.gbv.de
topic_facet	Hereditary sensory and autonomic neuropathies Molecular genetics Disease mechanisms Neurosciences. Biological psychiatry. Neuropsychiatry
isfreeaccess_bool	true
container_title	Neurobiology of Disease
authorswithroles_txt_mv	Nathalie Verpoorten @@aut@@ Peter De Jonghe @@aut@@ Vincent Timmerman @@aut@@
publishDateDaySort_date	2006-01-01T00:00:00Z
hierarchy_top_id	268125414
id	DOAJ069114382
language_de	englisch
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callnumber-first	R - Medicine
author	Nathalie Verpoorten
spellingShingle	Nathalie Verpoorten misc RC321-571 misc Hereditary sensory and autonomic neuropathies misc Molecular genetics misc Disease mechanisms misc Neurosciences. Biological psychiatry. Neuropsychiatry Disease mechanisms in hereditary sensory and autonomic neuropathies
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topic_title	RC321-571 Disease mechanisms in hereditary sensory and autonomic neuropathies Hereditary sensory and autonomic neuropathies Molecular genetics Disease mechanisms
topic	misc RC321-571 misc Hereditary sensory and autonomic neuropathies misc Molecular genetics misc Disease mechanisms misc Neurosciences. Biological psychiatry. Neuropsychiatry
topic_unstemmed	misc RC321-571 misc Hereditary sensory and autonomic neuropathies misc Molecular genetics misc Disease mechanisms misc Neurosciences. Biological psychiatry. Neuropsychiatry
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title	Disease mechanisms in hereditary sensory and autonomic neuropathies
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title_full	Disease mechanisms in hereditary sensory and autonomic neuropathies
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title_sort	disease mechanisms in hereditary sensory and autonomic neuropathies
callnumber	RC321-571
title_auth	Disease mechanisms in hereditary sensory and autonomic neuropathies
abstract	Inherited peripheral neuropathies are common monogenically inherited diseases of the peripheral nervous system. In the most common variant, i.e., the hereditary motor and sensory neuropathies, both motor and sensory nerves are affected. In contrast, sensory abnormalities predominate or are exclusively present in hereditary sensory and autonomic neuropathies (HSAN). HSAN are clinically and genetically heterogeneous and are subdivided according to mode of inheritance, age of onset and clinical evolution. In recent years, 6 disease-causing genes have been identified for autosomal dominant and recessive HSAN. However, vesicular transport and axonal trafficking seem important common pathways leading to degeneration of sensory and autonomic neurons. This review discusses the HSAN-related genes and their biological role in the disease mechanisms leading to HSAN.
abstractGer	Inherited peripheral neuropathies are common monogenically inherited diseases of the peripheral nervous system. In the most common variant, i.e., the hereditary motor and sensory neuropathies, both motor and sensory nerves are affected. In contrast, sensory abnormalities predominate or are exclusively present in hereditary sensory and autonomic neuropathies (HSAN). HSAN are clinically and genetically heterogeneous and are subdivided according to mode of inheritance, age of onset and clinical evolution. In recent years, 6 disease-causing genes have been identified for autosomal dominant and recessive HSAN. However, vesicular transport and axonal trafficking seem important common pathways leading to degeneration of sensory and autonomic neurons. This review discusses the HSAN-related genes and their biological role in the disease mechanisms leading to HSAN.
abstract_unstemmed	Inherited peripheral neuropathies are common monogenically inherited diseases of the peripheral nervous system. In the most common variant, i.e., the hereditary motor and sensory neuropathies, both motor and sensory nerves are affected. In contrast, sensory abnormalities predominate or are exclusively present in hereditary sensory and autonomic neuropathies (HSAN). HSAN are clinically and genetically heterogeneous and are subdivided according to mode of inheritance, age of onset and clinical evolution. In recent years, 6 disease-causing genes have been identified for autosomal dominant and recessive HSAN. However, vesicular transport and axonal trafficking seem important common pathways leading to degeneration of sensory and autonomic neurons. This review discusses the HSAN-related genes and their biological role in the disease mechanisms leading to HSAN.
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title_short	Disease mechanisms in hereditary sensory and autonomic neuropathies
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Disease mechanisms in hereditary sensory and autonomic neuropathies

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