C57BL/6JCya-Tmem106bem1/Cya
Common Name:
Tmem106b-KO
Product ID:
S-KO-18411
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
Quantity
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Basic Information
Strain Name
Tmem106b-KO
Strain ID
KOCMP-71900-Tmem106b-B6J-VB
Gene Name
Product ID
S-KO-18411
Gene Alias
2310036D22Rik; 5830455K21Rik; 6430519M21Rik
Background
C57BL/6JCya
NCBI ID
Modification
Conventional knockout
Chromosome
6
Phenotype
Document
Application
--
Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Tmem106bem1/Cya mice (Catalog S-KO-18411) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000031556
NCBI RefSeq
NM_027992
Target Region
Exon 4~5
Size of Effective Region
~3.2 kb
Detailed Document
Overview of Gene Research
TMEM106B, a lysosomal transmembrane protein, is crucial in regulating multiple aspects of lysosomal function [4]. It also plays a role in myelin lipid metabolism by interacting with galactosylceramidase [5]. Mutations in TMEM106B are risk factors for diverse neurodegenerative diseases [1].
Genetic variation in TMEM106B influences the risk and presentation of neurodegenerative diseases, especially frontotemporal dementia (FTD) caused by mutations in the progranulin gene (GRN) [6]. However, full deletion and partial reduction of TMEM106B in mouse and iPSC-derived human cell models of GRN deficiency did not reverse disease-associated phenotypes, suggesting that lowering TMEM106B levels may not be a viable therapeutic strategy for treating FTD-GRN [7].
In addition, TMEM106B has been found to form amyloid filaments in human brains in an age-dependent manner, which are present in various neurodegenerative diseases [3,6,8,9]. It can also serve as an alternative receptor for SARS-CoV-2 entry into angiotensin-converting enzyme 2 (ACE2)-negative cells [2].
In summary, TMEM106B is integral for lysosomal function and myelin lipid metabolism. Its role in neurodegenerative diseases, especially those related to GRN-associated FTD, has been studied using genetic models. The formation of amyloid filaments and its function as a viral entry receptor further highlight its significance in both neurological and infectious disease contexts.
References：
1. Jiao, Hai-Shan, Yuan, Peng, Yu, Jin-Tai. 2023. TMEM106B aggregation in neurodegenerative diseases: linking genetics to function. In Molecular neurodegeneration, 18, 54. doi:10.1186/s13024-023-00644-1. https://pubmed.ncbi.nlm.nih.gov/37563705/
2. Baggen, Jim, Jacquemyn, Maarten, Persoons, Leentje, Cherepanov, Peter, Daelemans, Dirk. 2023. TMEM106B is a receptor mediating ACE2-independent SARS-CoV-2 cell entry. In Cell, 186, 3427-3442.e22. doi:10.1016/j.cell.2023.06.005. https://pubmed.ncbi.nlm.nih.gov/37421949/
3. Schweighauser, Manuel, Arseni, Diana, Bacioglu, Mehtap, Goedert, Michel, Scheres, Sjors H W. 2022. Age-dependent formation of TMEM106B amyloid filaments in human brains. In Nature, 605, 310-314. doi:10.1038/s41586-022-04650-z. https://pubmed.ncbi.nlm.nih.gov/35344985/
4. Zhu, Min, Zhang, Guoxin, Meng, Lanxia, Fang, Xin, Zhang, Zhentao. 2024. Physiological and pathological functions of TMEM106B in neurodegenerative diseases. In Cellular and molecular life sciences : CMLS, 81, 209. doi:10.1007/s00018-024-05241-z. https://pubmed.ncbi.nlm.nih.gov/38710967/
5. Takahashi, Hideyuki, Perez-Canamas, Azucena, Lee, Chris W, Han, Xianlin, Strittmatter, Stephen M. 2024. Lysosomal TMEM106B interacts with galactosylceramidase to regulate myelin lipid metabolism. In Communications biology, 7, 1088. doi:10.1038/s42003-024-06810-5. https://pubmed.ncbi.nlm.nih.gov/39237682/
6. Perneel, Jolien, Neumann, Manuela, Heeman, Bavo, Rademakers, Rosa, Mackenzie, Ian R A. 2022. Accumulation of TMEM106B C-terminal fragments in neurodegenerative disease and aging. In Acta neuropathologica, 145, 285-302. doi:10.1007/s00401-022-02531-3. https://pubmed.ncbi.nlm.nih.gov/36527486/
7. Dominguez, Sara L, Laufer, Benjamin I, Ghosh, Arundhati Sengupta, Meilandt, William J, Easton, Amy. 2023. TMEM106B reduction does not rescue GRN deficiency in iPSC-derived human microglia and mouse models. In iScience, 26, 108362. doi:10.1016/j.isci.2023.108362. https://pubmed.ncbi.nlm.nih.gov/37965143/
8. Bacioglu, Mehtap, Schweighauser, Manuel, Gray, Derrick, Ghetti, Bernardino, Spillantini, Maria Grazia. 2024. Cleaved TMEM106B forms amyloid aggregates in central and peripheral nervous systems. In Acta neuropathologica communications, 12, 99. doi:10.1186/s40478-024-01813-z. https://pubmed.ncbi.nlm.nih.gov/38886865/
9. Chang, Andrew, Xiang, Xinyu, Wang, Jing, Mackenzie, Ian R A, Fitzpatrick, Anthony W P. 2022. Homotypic fibrillization of TMEM106B across diverse neurodegenerative diseases. In Cell, 185, 1346-1355.e15. doi:10.1016/j.cell.2022.02.026. https://pubmed.ncbi.nlm.nih.gov/35247328/
Quality Control Standard
Sperm Test
Pre-cryopreservation: Measurement of sperm concentration, determination of sperm viability.
Post-cryopreservation: A vial of cryopreserved sperms is selected for in-vitro fertilization from each batch.
Environmental Standards:SPF
Available Region:Global
Source:Cyagen