DOI: 10.5061/dryad.z08kprrpx
title: Pathogenic LRRK2 mutations cause loss of primary cilia and Neurturin in striatal Parvalbumin interneurons (mice stereology and human brain tile-scan)

description: Parkinson’s disease-associated, activating mutations in LRRK2 kinase block
 primary cilia formation in cell culture and in specific cell types in the
 brain. In the striatum which is important for movement control, about half
 of astrocytes and cholinergic interneurons, but not the predominant medium
 spiny neurons, lose their primary cilia. Here we show that Parvalbumin
 interneurons that are inhibitory regulators of movement also lose primary
 cilia. Without cilia, these neurons are not able to respond to Sonic
 Hedgehog signals that normally induce the expression of Patched RNA, and
 their numbers decrease. In addition, glial cell line-derived neurotrophic
 factor-related Neurturin RNA is significantly decreased. These experiments
 highlight the importance of Parvalbumin neurons in cilia-dependent,
 neuroprotective signaling pathways and show that LRRK2 activation
 correlates with decreased Neurturin production, resulting in less
 neuroprotection for dopamine neurons.
description: # Pathogenic LRRK2 mutations cause loss of primary cilia and Neurturin in
 striatal Parvalbumin interneurons (mice stereology and human brain
 tile-scan)
 [https://doi.org/10.5061/dryad.z08kprrpx](https://doi.org/10.5061/dryad.z08kprrpx) ## Description of the data and file structure This dataset contains the mice stereological microscopy and human tile-scan confocal images and the quantitation data of Parvalbumin neurons in the mice and human brains. ### Files and variables #### File: Fig.5\_Stereological\_microscopy.zip **Description:** This file contains 8 subfolders (Br4 to Br11) which have their corresponding stereological microscopy images (.jpx files) and .dat files. The name of each subfolder indicates the mouse ID. The file contains the Tabular data (.csv) and graphs in Prism that were used to generate graphs shown in Figure 5. Each variable (measurement) means a total estimated number of a certain category of a neuron (PV+ and cKit+ double positive, or PV+ alone) in a mouse brain (dorsal striatum). There are 4 mice in the wild-type (WT) group (Br8-11) and 4 mice in the LRRK2-R1441C (R1441C, RC) group (Br4-7). #### File: Fig6\_human\_PV\_numbers\_per\_square\_mm.zip **Description:** This file contains 3 subfolders (Control, G2019S, and sPD) which have their corresponding tile-scan microscopy images (.czi files). Control means healthy individual, G2019S means LRRK2-G2019S mutation Parkinson's patients, and sPD means sporadic Parkinson's patients. Their IDs were masked (de-identified) by special numbers that were given by Banner Sun Health Research Institute. The file contains the Tabular data (.csv) and graph in Prism that were used to generate graphs shown in Figure 6. Each variable (measurement) denotes an average number of PV neurons per area (square millimeter) in a human brain. There are 4 Controls, 4 sporadic Parkinson's patients, and 3 LRRK2-G2019S mutation Parkinson's patients data in this dataset. ## Code/software Microsoft Office (Excel) to open .csv files GraphPad Prism 9 (or newer) to open prism files. ZEISS ZEN (3.4 or newer) to open the .czi files (FIJI and ImageJ are also good). Stereo Investigator (v2024) to open .dat and .jpx files. ## Sharing/access information NA