Impaired intracellular Ca2+ signaling contributes to age-related cerebral small vessel disease in Col4a1 mutant mice

Humans and mice with mutations in COL4A1 and COL4A2 manifest hallmarks of cerebral small vessel disease (cSVD). Mice with a missense mutation in Col4a1 at amino acid 1344 (Col4a1+/G1344D) exhibit age-dependent intracerebral hemorrhage (ICH) and brain lesions. Here we report that this pathology was associated with the loss of myogenic vasoconstriction, an intrinsic vascular response essential for the autoregulation of cerebral blood flow. Electrophysiological analyses showed that the loss of myogenic constriction resulted from blunted pressure-induced smooth muscle cell (SMC) membrane depolarization. Further, we found that dysregulation of membrane potential was associated with impaired Ca2+-dependent activation of large-conductance Ca2+-activated K+ (BK) and transient receptor potential melastatin 4 (TRPM4) cation channels linked to disruptions in sarcoplasmic reticulum (SR) Ca2+ signaling.  Treating Col4a1+/G1344D mice with 4-phenylbutyrate, a compound that promotes the trafficking of ..., , , # Data set for \"Impaired intracellular Ca2+ signaling contributes to age-related cerebral small vessel disease in Col4a1 mutant mice\"

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This dataset contains all the processed data published in \"Impaired intracellular Ca2+ signaling contributes to age-related cerebral small vessel disease in Col4a1 mutant mice\".

## Description of the data and file structure

Figure_1_Processed_Data
* 1C: Percentage of brain area with Prussian blue staining in brain sections from 3 M-old Col4a1+/+ and Col4a1+/G1344D mice.
* 1E: Percentage of brain area with Prussian blue staining in brain sections from 12 M-old Col4a1+/+ and Col4a1+/G1344D mice.
* 1G: The number of brain lesions detected with high-field susceptibility-weighted magnetic resonance imaging of 12 M-old Col4a1+/+ and Col4a1+/G1344D mice.
* 1H: Total volume of brain lesions detected with high-field susceptibility-weighted magnetic resonance imaging of 12 M-old Col4a1+/+ and Col4a1+/G1344D mice.
* 1I: Localization of brain lesions det...