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-Tbc1d23em1flox/Cya
Common Name:
Tbc1d23-flox
Product ID:
S-CKO-13767
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
Quantity
Price:
Contact for Pricing
Basic Information
Strain Name
Tbc1d23-flox
Strain ID
CKOCMP-67581-Tbc1d23-B6J-VA
Gene Name
Tbc1d23
Product ID
S-CKO-13767
Gene Alias
4930451A13Rik; D030022P07Rik
Background
C57BL/6JCya
NCBI ID
67581
Modification
Conditional knockout
Chromosome
16
Phenotype
MGI:1914831
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-Tbc1d23em1flox/Cya mice (Catalog S-CKO-13767) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000023431
NCBI RefSeq
NM_026254
Target Region
Exon 3
Size of Effective Region
~1.5 kb
Detailed Document
Click here to download >>
Overview of Gene Research
Tbc1d23, a member of the Tre2-Bub2-Cdc16 (TBC) family, is a Golgi-localized protein. It plays a crucial role in endosome-to-Golgi cargo trafficking, acting as a bridge between Golgi-bound golgin-97/245 and the WASH/FAM21 complex on endosomal vesicles [3,4]. It is also involved in the LKB1-AMPK signaling axis at the Golgi, which controls cellular events like polarity, proliferation, and energy homeostasis [1]. Additionally, Tbc1d23 is associated with the pathogenesis of pontocerebellar hypoplasia (PCH), a neurodevelopment disorder [1,2,5,7,8].

In zebrafish models, depletion of Tbc1d23 leads to developmental defects similar to human PCH, including hindbrain volume loss, abnormal neuronal growth, and disrupted cortical neuron positioning [7,8]. Tbc1d23 deficiency can be rescued by Golgi-targeted expression of LKB1 in zebrafish, revealing its role in the LKB1-AMPK axis at the Golgi [1]. In mice, deletion of Tbc1d23 impairs the STING-IFN-I signaling, suggesting its role in innate immunity [6].

In conclusion, Tbc1d23 is essential for endosome-to-Golgi trafficking and the LKB1-AMPK signaling at the Golgi. Its study through animal models, especially zebrafish and mice, has provided insights into its role in PCH pathogenesis and innate immunity. Understanding Tbc1d23 functions may offer new perspectives for treating related neurodevelopmental disorders and immune-related conditions.

References:
1. Tu, Yingfeng, Yang, Qin, Tang, Min, Dai, Lunzhi, Jia, Da. 2024. TBC1D23 mediates Golgi-specific LKB1 signaling. In Nature communications, 15, 1785. doi:10.1038/s41467-024-46166-2. https://pubmed.ncbi.nlm.nih.gov/38413626/
2. Zhao, Lin, Deng, Huaqing, Yang, Qing, Billadeau, Daniel D, Jia, Da. 2023. FAM91A1-TBC1D23 complex structure reveals human genetic variations susceptible for PCH. In Proceedings of the National Academy of Sciences of the United States of America, 120, e2309910120. doi:10.1073/pnas.2309910120. https://pubmed.ncbi.nlm.nih.gov/37903274/
3. Liu, Dingdong, Yang, Fan, Liu, Zhe, Mo, Xianming, Jia, Da. 2020. Structure of TBC1D23 N-terminus reveals a novel role for rhodanese domain. In PLoS biology, 18, e3000746. doi:10.1371/journal.pbio.3000746. https://pubmed.ncbi.nlm.nih.gov/32453802/
4. Shin, John J H, Gillingham, Alison K, Begum, Farida, Chadwick, Jessica, Munro, Sean. 2017. TBC1D23 is a bridging factor for endosomal vesicle capture by golgins at the trans-Golgi. In Nature cell biology, 19, 1424-1432. doi:10.1038/ncb3627. https://pubmed.ncbi.nlm.nih.gov/29084197/
5. Huang, Wenjie, Liu, Zhe, Yang, Fan, Mo, Xianming, Jia, Da. 2019. Structural and functional studies of TBC1D23 C-terminal domain provide a link between endosomal trafficking and PCH. In Proceedings of the National Academy of Sciences of the United States of America, 116, 22598-22608. doi:10.1073/pnas.1909316116. https://pubmed.ncbi.nlm.nih.gov/31624125/
6. Wang, Jinrui, Niu, Shenghui, Hu, Xiao, Billadeau, Daniel D, Jia, Da. 2025. Trans-Golgi network tethering factors regulate TBK1 trafficking and promote the STING-IFN-I pathway. In Cell discovery, 11, 23. doi:10.1038/s41421-024-00763-z. https://pubmed.ncbi.nlm.nih.gov/40097395/
7. Marin-Valencia, Isaac, Gerondopoulos, Andreas, Zaki, Maha S, Barr, Francis A, Gleeson, Joseph G. 2017. Homozygous Mutations in TBC1D23 Lead to a Non-degenerative Form of Pontocerebellar Hypoplasia. In American journal of human genetics, 101, 441-450. doi:10.1016/j.ajhg.2017.07.015. https://pubmed.ncbi.nlm.nih.gov/28823706/
8. Ivanova, Ekaterina L, Mau-Them, Frédéric Tran, Riazuddin, Saima, van Bokhoven, Hans, Chelly, Jamel. 2017. Homozygous Truncating Variants in TBC1D23 Cause Pontocerebellar Hypoplasia and Alter Cortical Development. In American journal of human genetics, 101, 428-440. doi:10.1016/j.ajhg.2017.07.010. https://pubmed.ncbi.nlm.nih.gov/28823707/
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