Expression of TMEM106B, the frontotemporal lobar degeneration-associated protein, in normal and diseased human brain.

Background: Frontotemporal lobar degeneration (FTLD) is the second most common cause of dementia in individuals under 65 years old and manifests as alterations in behavior, personality, or language secondary to degeneration of the frontal and/or temporal lobes. FTLD-TDP, the largest neuropathological subset of FTLD, is characterized by hyperphosphorylated, ubiquitinated TAR DNA-binding protein 43 (TDP-43) inclusions. Mutations in progranulin (GRN), a neuroprotective growth factor, are one of the most common Mendelian genetic causes of FTLD-TDP.

Clinical and biochemical differences in patients having Parkinson disease with vs without GBA mutations.

Importance: Biochemical abnormalities present in GBA (mut/wt) carriers may offer new pathogenetic insights to and potential therapeutic targets in Parkinson disease (PD).

Objective: To determine whether patients having PD with vs without GBA mutations differ in clinical phenotype or plasma protein expression.

Design And Setting: Case-control study of patients having PD with vs without GBA mutations.

Risk genotypes at TMEM106B are associated with cognitive impairment in amyotrophic lateral sclerosis.

TMEM106B has recently been identified as a genetic risk factor for frontotemporal lobar degeneration with TDP-43 inclusions (FTLD-TDP). Amyotrophic lateral sclerosis (ALS), like FTLD-TDP, is characterized by pathological TDP-43 inclusions. We therefore investigated whether FTLD-TDP-associated risk genotypes at TMEM106B (1) contribute to risk of developing ALS or (2) modify the clinical presentation in ALS.

Effect of image orientation and size on object recognition: responses of single units in the macaque monkey temporal cortex.

This study examined how cells in the temporal cortex code orientation and size of a complex object. The study focused on cells selectively responsive to the sight of the head and body but unresponsive to control stimuli. The majority of cells tested (19/26, 73%) were selectively responsive to a particular orientation in the picture plane of the static whole body stimulus, 7/26 cells showed generalisation responding to all orientations (three cells with orientation tuning superimposed on a generalised response).

Does parietal cortex contribute to feature binding?

We studied the involvement of the right parietal cortex in visual conjunction search, where two features are present in the array and spatial attention and feature binding is required, and in subset search, where two features are also present but only one of them is needed in order to group stimuli together (the subset) and allow parallel processing without the need for feature binding. Six patients with right parietal lobe lesions, six age-matched controls, and three control patients with left parietal lesions were tested on these two tasks. Patients with right parietal lesions were significantly slower than normal controls in the conjunction task, especially for target-absent trials.