TABLE 3.

Characteristics of pTDP-43 Expression in Neurodegenerative Diseases

Disease (Protein)Author (References)Number of PatientsProportion of Patients With pTDP-43 Expression (%)Brain Distribution of pTDP-43Morphology of pTDP-43Association With Disease ProteinpTDP-43 Expression Type
PD, DLB (α-synuclein)Yasuda et al (17)1010Hippocampus, entorhinalNCIColocalizaion(+)Aging type
Higashi et al (18)1145Amygdala, hippocampus, entorhinalNCI, DNAging type
Arai et al (19)1553Amygdala, hippocampus, entorhinalNCI, DNColocalization(+)Aging type
Uchino et al (20)1172.7Hippocampus, amygdala, medullaDN, NCI, GCIAging type
McAleese et al (21)1533.3Amygdala, hippocampusNCIAging type
Buciuc et al (22)39537Hippocampusnot examined
Uemura et al (23)31321Amygdala, hippocampus, entorhinal (LATE)NCIAging type
Guam PDC (tau)Hasegawa et al (24)6100HippocampusNCI, DNAging type
Geser et al (25)2646.2Amygdala, hippocampus, entorhinalNCI, GCIAging type
Miklossy et al (26)35100Amygdala, hippocampus, entorhinalNCIAging type
PSP (tau)King et al (27)520NeocortexNCIAging type
Yokota et al (12)1926Amygdala, hippocampus, entorhinalNCIColocalization(+)Aging type
Kertesz et al (28)10None
Koga et al (29)9456Amygdala, hippocampus, PSP lesions (substantia nigra, subthalamic nucleus, globus pallidus)NCI, DN, NIINeurodegenerative type
CBD (tau)Uryu et al (30)3915.4Hippocampus, entorhinal, temporal-frontal cortexDNAging type
Yokota et al (12)1217Amygdala, hippocampus, entorhinalNCIColocalization(−)Aging type
Kouri et al (31)1100Olivary nucleusNCI
Yamashita et al (32)1100CBD lesions (cerebral cortex, pons, medulla oblongata)NCIColocalization(+)Neurodegenerative type
Koga et al (33)18745CBD lesions (midbrain tegmentum, subthalamic nucleus, pontine tegmentum, substantia nigra)NCINeurodegenerative type
Sainouchi et al (34)1090CBD lesions (putamen, globus pallidus, thalamus, subthalamic nucleus, pontine tegmentum)NCI, GCI, DNColocalization(+)Neurodegenerative type
MSA (α-synuclein)Geser et al (35)29rareAmygdala, hippocampus, entorhinalDN, GCI, NCIAging type
Sousa et al (36)10None
Koga et al (37)1867Amygdala, hippocampusGCIColocalization(+)Aging type
Nwabuobi et al (38)1100Lenticular nucleus, paracentral cortexNCI, GCIColocalization(+)Aging type
AGD (tau)Fujishiro et al (39)1560Amygdala, hippocampus, entorhinalNCI, GCIAging type
Uchino et al (20)1154.5Hippocampus, amygdalaDN, NCI, GCIAging type
SCA2Toyoshima et al (14)1100SCA2 lesions (motor cortex, thalamus, red nucleus, pons, inferior olive)NCINeurodegenerative type
SCA3Tan et al (40)10100Cranial nucleus, spinal cordNCINeurodegenerative type
Huntington (polyglutamine)Schwab et al (41)10100Huntington lesions (frontal and temporal cortex)NCI, DNColocalization(+)Neurodegenerative type
Tada et al (42)4100Huntington lesions (cerebral cortex, thalamus, hypoglossal nucleus, anterior horn)NCI, DNColocalization(−)Neurodegenerative type
Disease (Protein)Author (References)Number of PatientsProportion of Patients With pTDP-43 Expression (%)Brain Distribution of pTDP-43Morphology of pTDP-43Association With Disease ProteinpTDP-43 Expression Type
PD, DLB (α-synuclein)Yasuda et al (17)1010Hippocampus, entorhinalNCIColocalizaion(+)Aging type
Higashi et al (18)1145Amygdala, hippocampus, entorhinalNCI, DNAging type
Arai et al (19)1553Amygdala, hippocampus, entorhinalNCI, DNColocalization(+)Aging type
Uchino et al (20)1172.7Hippocampus, amygdala, medullaDN, NCI, GCIAging type
McAleese et al (21)1533.3Amygdala, hippocampusNCIAging type
Buciuc et al (22)39537Hippocampusnot examined
Uemura et al (23)31321Amygdala, hippocampus, entorhinal (LATE)NCIAging type
Guam PDC (tau)Hasegawa et al (24)6100HippocampusNCI, DNAging type
Geser et al (25)2646.2Amygdala, hippocampus, entorhinalNCI, GCIAging type
Miklossy et al (26)35100Amygdala, hippocampus, entorhinalNCIAging type
PSP (tau)King et al (27)520NeocortexNCIAging type
Yokota et al (12)1926Amygdala, hippocampus, entorhinalNCIColocalization(+)Aging type
Kertesz et al (28)10None
Koga et al (29)9456Amygdala, hippocampus, PSP lesions (substantia nigra, subthalamic nucleus, globus pallidus)NCI, DN, NIINeurodegenerative type
CBD (tau)Uryu et al (30)3915.4Hippocampus, entorhinal, temporal-frontal cortexDNAging type
Yokota et al (12)1217Amygdala, hippocampus, entorhinalNCIColocalization(−)Aging type
Kouri et al (31)1100Olivary nucleusNCI
Yamashita et al (32)1100CBD lesions (cerebral cortex, pons, medulla oblongata)NCIColocalization(+)Neurodegenerative type
Koga et al (33)18745CBD lesions (midbrain tegmentum, subthalamic nucleus, pontine tegmentum, substantia nigra)NCINeurodegenerative type
Sainouchi et al (34)1090CBD lesions (putamen, globus pallidus, thalamus, subthalamic nucleus, pontine tegmentum)NCI, GCI, DNColocalization(+)Neurodegenerative type
MSA (α-synuclein)Geser et al (35)29rareAmygdala, hippocampus, entorhinalDN, GCI, NCIAging type
Sousa et al (36)10None
Koga et al (37)1867Amygdala, hippocampusGCIColocalization(+)Aging type
Nwabuobi et al (38)1100Lenticular nucleus, paracentral cortexNCI, GCIColocalization(+)Aging type
AGD (tau)Fujishiro et al (39)1560Amygdala, hippocampus, entorhinalNCI, GCIAging type
Uchino et al (20)1154.5Hippocampus, amygdalaDN, NCI, GCIAging type
SCA2Toyoshima et al (14)1100SCA2 lesions (motor cortex, thalamus, red nucleus, pons, inferior olive)NCINeurodegenerative type
SCA3Tan et al (40)10100Cranial nucleus, spinal cordNCINeurodegenerative type
Huntington (polyglutamine)Schwab et al (41)10100Huntington lesions (frontal and temporal cortex)NCI, DNColocalization(+)Neurodegenerative type
Tada et al (42)4100Huntington lesions (cerebral cortex, thalamus, hypoglossal nucleus, anterior horn)NCI, DNColocalization(−)Neurodegenerative type

PD, Parkinson disease; DLB, Dementia with Lewy bodies; Guam PDC, Guam parkinsonism-dementia complex; PSP, progressive supranuclear palsy; CBD, cortico-basal degeneration; MSA, multiple system atrophy; AGD, argyrophilic grain disease; SCA2, spinocerebellar ataxia type 2; SCA3, spinocerebellar ataxia type 3; NCI, neuronal cytoplasmic inclusion; GCI, oligodendroglial cytoplasmic inclusion; DN, dystrophic neurite.

TABLE 3.

Characteristics of pTDP-43 Expression in Neurodegenerative Diseases

Disease (Protein)Author (References)Number of PatientsProportion of Patients With pTDP-43 Expression (%)Brain Distribution of pTDP-43Morphology of pTDP-43Association With Disease ProteinpTDP-43 Expression Type
PD, DLB (α-synuclein)Yasuda et al (17)1010Hippocampus, entorhinalNCIColocalizaion(+)Aging type
Higashi et al (18)1145Amygdala, hippocampus, entorhinalNCI, DNAging type
Arai et al (19)1553Amygdala, hippocampus, entorhinalNCI, DNColocalization(+)Aging type
Uchino et al (20)1172.7Hippocampus, amygdala, medullaDN, NCI, GCIAging type
McAleese et al (21)1533.3Amygdala, hippocampusNCIAging type
Buciuc et al (22)39537Hippocampusnot examined
Uemura et al (23)31321Amygdala, hippocampus, entorhinal (LATE)NCIAging type
Guam PDC (tau)Hasegawa et al (24)6100HippocampusNCI, DNAging type
Geser et al (25)2646.2Amygdala, hippocampus, entorhinalNCI, GCIAging type
Miklossy et al (26)35100Amygdala, hippocampus, entorhinalNCIAging type
PSP (tau)King et al (27)520NeocortexNCIAging type
Yokota et al (12)1926Amygdala, hippocampus, entorhinalNCIColocalization(+)Aging type
Kertesz et al (28)10None
Koga et al (29)9456Amygdala, hippocampus, PSP lesions (substantia nigra, subthalamic nucleus, globus pallidus)NCI, DN, NIINeurodegenerative type
CBD (tau)Uryu et al (30)3915.4Hippocampus, entorhinal, temporal-frontal cortexDNAging type
Yokota et al (12)1217Amygdala, hippocampus, entorhinalNCIColocalization(−)Aging type
Kouri et al (31)1100Olivary nucleusNCI
Yamashita et al (32)1100CBD lesions (cerebral cortex, pons, medulla oblongata)NCIColocalization(+)Neurodegenerative type
Koga et al (33)18745CBD lesions (midbrain tegmentum, subthalamic nucleus, pontine tegmentum, substantia nigra)NCINeurodegenerative type
Sainouchi et al (34)1090CBD lesions (putamen, globus pallidus, thalamus, subthalamic nucleus, pontine tegmentum)NCI, GCI, DNColocalization(+)Neurodegenerative type
MSA (α-synuclein)Geser et al (35)29rareAmygdala, hippocampus, entorhinalDN, GCI, NCIAging type
Sousa et al (36)10None
Koga et al (37)1867Amygdala, hippocampusGCIColocalization(+)Aging type
Nwabuobi et al (38)1100Lenticular nucleus, paracentral cortexNCI, GCIColocalization(+)Aging type
AGD (tau)Fujishiro et al (39)1560Amygdala, hippocampus, entorhinalNCI, GCIAging type
Uchino et al (20)1154.5Hippocampus, amygdalaDN, NCI, GCIAging type
SCA2Toyoshima et al (14)1100SCA2 lesions (motor cortex, thalamus, red nucleus, pons, inferior olive)NCINeurodegenerative type
SCA3Tan et al (40)10100Cranial nucleus, spinal cordNCINeurodegenerative type
Huntington (polyglutamine)Schwab et al (41)10100Huntington lesions (frontal and temporal cortex)NCI, DNColocalization(+)Neurodegenerative type
Tada et al (42)4100Huntington lesions (cerebral cortex, thalamus, hypoglossal nucleus, anterior horn)NCI, DNColocalization(−)Neurodegenerative type
Disease (Protein)Author (References)Number of PatientsProportion of Patients With pTDP-43 Expression (%)Brain Distribution of pTDP-43Morphology of pTDP-43Association With Disease ProteinpTDP-43 Expression Type
PD, DLB (α-synuclein)Yasuda et al (17)1010Hippocampus, entorhinalNCIColocalizaion(+)Aging type
Higashi et al (18)1145Amygdala, hippocampus, entorhinalNCI, DNAging type
Arai et al (19)1553Amygdala, hippocampus, entorhinalNCI, DNColocalization(+)Aging type
Uchino et al (20)1172.7Hippocampus, amygdala, medullaDN, NCI, GCIAging type
McAleese et al (21)1533.3Amygdala, hippocampusNCIAging type
Buciuc et al (22)39537Hippocampusnot examined
Uemura et al (23)31321Amygdala, hippocampus, entorhinal (LATE)NCIAging type
Guam PDC (tau)Hasegawa et al (24)6100HippocampusNCI, DNAging type
Geser et al (25)2646.2Amygdala, hippocampus, entorhinalNCI, GCIAging type
Miklossy et al (26)35100Amygdala, hippocampus, entorhinalNCIAging type
PSP (tau)King et al (27)520NeocortexNCIAging type
Yokota et al (12)1926Amygdala, hippocampus, entorhinalNCIColocalization(+)Aging type
Kertesz et al (28)10None
Koga et al (29)9456Amygdala, hippocampus, PSP lesions (substantia nigra, subthalamic nucleus, globus pallidus)NCI, DN, NIINeurodegenerative type
CBD (tau)Uryu et al (30)3915.4Hippocampus, entorhinal, temporal-frontal cortexDNAging type
Yokota et al (12)1217Amygdala, hippocampus, entorhinalNCIColocalization(−)Aging type
Kouri et al (31)1100Olivary nucleusNCI
Yamashita et al (32)1100CBD lesions (cerebral cortex, pons, medulla oblongata)NCIColocalization(+)Neurodegenerative type
Koga et al (33)18745CBD lesions (midbrain tegmentum, subthalamic nucleus, pontine tegmentum, substantia nigra)NCINeurodegenerative type
Sainouchi et al (34)1090CBD lesions (putamen, globus pallidus, thalamus, subthalamic nucleus, pontine tegmentum)NCI, GCI, DNColocalization(+)Neurodegenerative type
MSA (α-synuclein)Geser et al (35)29rareAmygdala, hippocampus, entorhinalDN, GCI, NCIAging type
Sousa et al (36)10None
Koga et al (37)1867Amygdala, hippocampusGCIColocalization(+)Aging type
Nwabuobi et al (38)1100Lenticular nucleus, paracentral cortexNCI, GCIColocalization(+)Aging type
AGD (tau)Fujishiro et al (39)1560Amygdala, hippocampus, entorhinalNCI, GCIAging type
Uchino et al (20)1154.5Hippocampus, amygdalaDN, NCI, GCIAging type
SCA2Toyoshima et al (14)1100SCA2 lesions (motor cortex, thalamus, red nucleus, pons, inferior olive)NCINeurodegenerative type
SCA3Tan et al (40)10100Cranial nucleus, spinal cordNCINeurodegenerative type
Huntington (polyglutamine)Schwab et al (41)10100Huntington lesions (frontal and temporal cortex)NCI, DNColocalization(+)Neurodegenerative type
Tada et al (42)4100Huntington lesions (cerebral cortex, thalamus, hypoglossal nucleus, anterior horn)NCI, DNColocalization(−)Neurodegenerative type

PD, Parkinson disease; DLB, Dementia with Lewy bodies; Guam PDC, Guam parkinsonism-dementia complex; PSP, progressive supranuclear palsy; CBD, cortico-basal degeneration; MSA, multiple system atrophy; AGD, argyrophilic grain disease; SCA2, spinocerebellar ataxia type 2; SCA3, spinocerebellar ataxia type 3; NCI, neuronal cytoplasmic inclusion; GCI, oligodendroglial cytoplasmic inclusion; DN, dystrophic neurite.

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