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-Ctnsem1flox/Cya
Common Name:
Ctns-flox
Product ID:
S-CKO-17042
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
Quantity
Price:
Contact for Pricing
Basic Information
Strain Name
Ctns-flox
Strain ID
CKOCMP-83429-Ctns-B6J-VA
Gene Name
Ctns
Product ID
S-CKO-17042
Gene Alias
-
Background
C57BL/6JCya
NCBI ID
83429
Modification
Conditional knockout
Chromosome
11
Phenotype
MGI:1932872
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-Ctnsem1flox/Cya mice (Catalog S-CKO-17042) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000108476
NCBI RefSeq
NM_001357891
Target Region
Exon 4
Size of Effective Region
~0.6 kb
Detailed Document
Click here to download >>
Overview of Gene Research
Ctns, encoding the lysosomal membrane protein cystinosin, is crucial as it transports cystine out of lysosomes into the cytoplasm [1,2,3,4]. Mutations in Ctns lead to cystinosis, an autosomal recessive lysosomal storage disorder [1,2,3,4]. The associated disruption in cystine transport results in intra-lysosomal cystine accumulation in all body cells and organs, with the kidneys often being initially affected [2].

Cystinosis has different forms related to Ctns mutations. Nephropathic or infantile type, the most common, is characterized by renal failure at around 10 years of age and other systemic complications, associated with 32 different Ctns mutations [1]. Intermediate cystinosis, with later-onset renal disease, is linked to three different mutations, and benign or non-nephropathic cystinosis, presenting with corneal crystals and photophobia, is associated with two other mutations [1]. In general, only certain splicing or missense mutations are related to milder phenotypes [1]. More than 140 Ctns mutations have been reported worldwide [3]. Different studies have identified various mutations in different populations. For example, in Iranian nephropathic cystinosis patients, mutations were found in exons 6 and 7 in 50% of cases, and novel mutations were also detected [5]. In Tunisian patients with ocular cystinosis, 7 mutations were disclosed, including missense, duplication, frameshift, and splice-site mutations [6].

In conclusion, Ctns is essential for normal cystine transport from lysosomes. Studies on Ctns-related cystinosis, through identification of various mutations in different populations, help understand the molecular basis of the disease. This knowledge is fundamental for developing new therapeutic strategies, such as targeted gene therapy, to address the kidney and extra-renal phenotypes of cystinosis [3].

References:
1. Anikster, Y, Shotelersuk, V, Gahl, W A. . CTNS mutations in patients with cystinosis. In Human mutation, 14, 454-8. doi:. https://pubmed.ncbi.nlm.nih.gov/10571941/
2. Elmonem, Mohamed A, Veys, Koenraad R, Soliman, Neveen A, van den Heuvel, Lambertus P, Levtchenko, Elena. 2016. Cystinosis: a review. In Orphanet journal of rare diseases, 11, 47. doi:10.1186/s13023-016-0426-y. https://pubmed.ncbi.nlm.nih.gov/27102039/
3. David, Dries, Princiero Berlingerio, Sante, Elmonem, Mohamed A, Gijsbers, Rik, Levtchenko, Elena. 2018. Molecular Basis of Cystinosis: Geographic Distribution, Functional Consequences of Mutations in the CTNS Gene, and Potential for Repair. In Nephron, 141, 133-146. doi:10.1159/000495270. https://pubmed.ncbi.nlm.nih.gov/30554218/
4. Hohenfellner, Katharina, Zerell, Kirstin, Haffner, Dieter. 2023. Cystinosis. In Klinische Monatsblatter fur Augenheilkunde, 240, 251-259. doi:10.1055/a-2022-8522. https://pubmed.ncbi.nlm.nih.gov/36977426/
5. Ghazi, Farideh, Hosseini, Rozita, Akouchekian, Mansoureh, Gahl, William A, Behnam, Babak. 2017. CTNS molecular genetics profile in a Persian nephropathic cystinosis population. In Nefrologia : publicacion oficial de la Sociedad Espanola Nefrologia, 37, 301-310. doi:10.1016/j.nefro.2016.11.024. https://pubmed.ncbi.nlm.nih.gov/28238446/
6. Chkioua, Latifa, Amri, Yessine, Saheli, Chaima, Ghorbel, Mohamed, Laradi, Sandrine. 2022. Molecular characterization of CTNS mutations in Tunisian patients with ocular cystinosis. In Diagnostic pathology, 17, 44. doi:10.1186/s13000-022-01221-8. https://pubmed.ncbi.nlm.nih.gov/35524314/
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