Logo
Homepage
Explore Our Models
My Cart
Contact
Subscribe
Models
Genetically Engineered Animals
Knockout Mice
Knockout Rats
Knockin Mice
Knockin Rats
Transgenic Mice
Transgenic Rats
Model Generation Techniques
Turboknockout<sup>®</sup> Gene Targeting
ES Cell Gene Targeting
Targeted Gene Editing
Regular Transgenic
PiggyBac Transgenesis
BAC Transgenic
Research Models
HUGO-GT™ Humanized Mice
Cre Mouse Lines
Humanized Target Gene Models
Metabolic Disease Models
Ophthalmic Disease Models
Neurological Disease Models
Autoimmune Disease Models
Immunodeficient Mouse Models
Humanized Immune System Mouse Models
Oncology & Immuno-oncology Models
Covid-19 Mouse Models
MouseAtlas Model Library
Knockout Cell Line Product Catalog
Tumor Cell Line Product Catalog
AAV Standard Product Catalog
Animal Supporting Services
Breeding Services
Cryopreservation & Recovery
Phenotyping Services
BAC Modification
Custom Cell Line Models
Induced Pluripotent Stem Cells (iPSCs)
Knockout Cell Lines
Knockin Cell Lines
Point Mutation Cell Lines
Overexpression Cell Lines
Virus Packaging
Adeno-associated Virus (AAV) Packaging
Lentivirus Packaging
Adenovirus Packaging
CRO Services
By Therapeutic Area
Oncology
Ophthalmology
Neuroscience
Metabolic & Cardiovascular Diseases
Autoimmune & Inflammatory
By Drug Type
AI-Powered AAV Discovery
Gene Therapy
Oligonucleotide Therapy
Antibody Therapy
Cell Immunotherapy
Resources
Promotion
Events & Webinars
Newsroom
Blogs & Insights
Resource Vault
Reference Databases
Peer-Reviewed Citations
Rare Disease Data Center
AbSeek
Cell iGeneEditor™ System
OriCell
Quality
Facility Overview
Animal Health & Welfare
Health Reports
About Us
Corporate Overview
Our Partners
Careers
Contact Us
Login
Request a Product Quote
Select products from our catalogs and submit your request. Our team will get back to you with detailed information.
Full Name
Email
Phone Number
Organization
Job Role
Country
Catalog Type
Product Name
Additional Comments
Cyagen values your privacy. We’d like to keep you informed about our latest offerings and insights. Your preferences:
You may unsubscribe from these communications at any time. See our Privacy Policy for details on opting out and data protection.
By clicking the button below, you consent to allow Cyagen to store and process the personal information submitted in this form to provide you the content requested.
C57BL/6JCya-Tnnt1em1flox/Cya
Common Name:
Tnnt1-flox
Product ID:
S-CKO-18372
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
Quantity
Price:
Contact for Pricing
Basic Information
Strain Name
Tnnt1-flox
Strain ID
CKOCMP-21955-Tnnt1-B6J-VB
Gene Name
Tnnt1
Product ID
S-CKO-18372
Gene Alias
Tnt; sTnT; ssTnT
Background
C57BL/6JCya
NCBI ID
21955
Modification
Conditional knockout
Chromosome
7
Phenotype
MGI:1333868
Document
Click here to download >>
Application
--
More
Rare Disease Data Center >>
Note
Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Tnnt1em1flox/Cya mice (Catalog S-CKO-18372) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000108587
NCBI RefSeq
NM_001277903
Target Region
Exon 7~9
Size of Effective Region
~1.6 kb
Detailed Document
Click here to download >>
Overview of Gene Research
Tnnt1, encoding slow skeletal muscle troponin T, is one of three homologous genes in vertebrates that encode muscle type-specific troponin T isoforms. Troponin T (TnT) is crucial for the calcium regulation of actin thin filament function and essential for striated muscle contraction [2].

In terms of disease-related findings, TNNT1-related nemaline myopathies are clinically and genetically heterogeneous. For example, a 16-year-old Korean boy with progressive muscle weakness was found to have novel compound heterozygous mutations in TNNT1, and exon 11 skipping in the splicing variant was confirmed. The functional impact of the missense variant was verified using a zebrafish loss-of-function model [1]. Also, the 'Amish' nemaline myopathy, caused by a TNNT1 pathogenic mutation, is an infantile-onset, lethal disease. Affected children show progressive muscle weakness, atrophy, and respiratory failure. Similar phenotypes were observed in two transgenic murine models (Tnnt1-/-and Tnnt1 c.505G>T) [3]. A novel recessive congenital TNNT1 core-rod myopathy was reported in French Canadians. A zebrafish loss-of-function model was created to assess the pathogenicity of the identified variant, and wild-type TNNT1 mRNA could rescue the zebrafish morphants while mutant transcripts failed [4]. In a Chinese girl with TNNT1 nemaline myopathy, a splicing mutation led to exon skipping and both the truncation and splicing mutations triggered nonsense-mediated mRNA decay [5].

In conclusion, Tnnt1 is essential for normal skeletal muscle function. Studies using gene knockout or loss-of-function models, such as zebrafish and transgenic murine models, have significantly contributed to understanding TNNT1-related nemaline myopathies. These models help reveal the role of Tnnt1 in muscle development and function, providing insights into potential therapeutic strategies for these muscle-related disorders.

References:
1. Lee, Seungbok, Eum, Juneyong, Park, Soojin, Kee, Yun, Chae, Jong Hee. 2021. TNNT1 myopathy with novel compound heterozygous mutations. In Neuromuscular disorders : NMD, 32, 176-184. doi:10.1016/j.nmd.2021.12.003. https://pubmed.ncbi.nlm.nih.gov/35165004/
2. Wei, Bin, Jin, J-P. 2016. TNNT1, TNNT2, and TNNT3: Isoform genes, regulation, and structure-function relationships. In Gene, 582, 1-13. doi:10.1016/j.gene.2016.01.006. https://pubmed.ncbi.nlm.nih.gov/26774798/
3. Fox, Michael D, Carson, Vincent J, Feng, Han-Zhong, Jin, J-P, Strauss, Kevin A. . TNNT1 nemaline myopathy: natural history and therapeutic frontier. In Human molecular genetics, 27, 3272-3282. doi:10.1093/hmg/ddy233. https://pubmed.ncbi.nlm.nih.gov/29931346/
4. Pellerin, David, Aykanat, Asli, Ellezam, Benjamin, Brais, Bernard, Chrestian, Nicolas. 2020. Novel Recessive TNNT1 Congenital Core-Rod Myopathy in French Canadians. In Annals of neurology, 87, 568-583. doi:10.1002/ana.25685. https://pubmed.ncbi.nlm.nih.gov/31970803/
5. Wang, Guangyu, Zhao, Dandan, Yan, Chuanzhu, Lin, Pengfei. 2022. Exon skipping caused by splicing mutation in TNNT1 nemaline myopathy. In Journal of human genetics, 68, 97-101. doi:10.1038/s10038-022-01096-z. https://pubmed.ncbi.nlm.nih.gov/36446828/
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
Model Library
Model Library
Resources
Resources
Animal Quality
Animal Quality
Get Support
Get Support
Address:
2255 Martin Avenue, Suite E Santa Clara, CA 95050-2709, US
Tel:
800-921-8930 (8-6pm PST)
+1408-963-0306 (lnt’l)
Fax:
408-969-0338
Email:
animal-service@cyagen.com
service@cyagen.us
CRO Services
OncologyOphthalmologyNeuroscienceMetabolic & CardiovascularAutoimmune & InflammatoryGene TherapyAntibody Therapy
About Us
Corporate OverviewOur PartnersCareersContact Us
Social Media
Disclaimer: Pricing and availability of our products and services vary by region. Listed prices are applicable to the specific countries. Please contact us for more information.
Copyright © 2025 Cyagen. All rights reserved.
Privacy Policy
Site Map
Stay Updated with the Latest from Cyagen
Get the latest news on our research models, CRO services, scientific resources, and special offers—tailored to your research needs and delivered straight to your inbox.
Full Name
Email
Organization
Country
Areas of Interest