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C57BL/6JCya-Gaaem1flox/Cya
Common Name:
Gaa-flox
Product ID:
S-CKO-18952
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
Quantity
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Basic Information
Strain Name
Gaa-flox
Strain ID
CKOCMP-14387-Gaa-B6J-VB
Gene Name
Gaa
Product ID
S-CKO-18952
Gene Alias
E430018M07Rik
Background
C57BL/6JCya
NCBI ID
14387
Modification
Conditional knockout
Chromosome
11
Phenotype
MGI:95609
Document
Click here to download >>
Application
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More
Rare Disease Data Center >>
Note
Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Gaaem1flox/Cya mice (Catalog S-CKO-18952) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000026666
NCBI RefSeq
NM_001159324
Target Region
Exon 4~8
Size of Effective Region
~1.8 kb
Detailed Document
Click here to download >>
Overview of Gene Research
Gaa, also known as acid alpha-1,4-glucosidase, is a gene encoding an enzyme that catalyzes the hydrolysis of α-1,4 and α-1,6-glucosidic bonds of glycogen. It is involved in the lysosomal breakdown of glycogen, which is crucial for normal cellular function. Mutations in the Gaa gene are associated with Pompe disease, an autosomal recessive disorder [1].

In a mouse model of Pompe disease, the Gaa-/-mouse, Gaa deficiency leads to glycogen accumulation in distal airway cells, disrupting surfactant homeostasis and contributing to respiratory impairments. Histological analysis showed increased accumulation of lysosomal-associated membrane protein 1 in the lungs, ultrastructural examination revealed intracytoplasmic vacuoles enlargement and lamellar body engorgement, and transcriptomic analysis demonstrated dysregulation of surfactant proteins in alveolar type 2 cells [2]. Also, knockdown experiments in zebrafish and human umbilical vein endothelial cells (HUVECs) showed that Gaa deficiency promotes angiogenesis. The mechanism is related to autophagy disorder, which causes up-regulation of Rac1, a small GTPase [3].

In conclusion, Gaa is essential for normal glycogen metabolism. Studies using Gaa-deficient mouse models have revealed its significance in respiratory function and angiogenesis, providing insights into the pathogenesis of Pompe disease. Understanding the role of Gaa in these biological processes may contribute to the development of new treatment strategies for Pompe disease and related conditions.

References:
1. Taverna, Simona, Cammarata, Giuseppe, Colomba, Paolo, Vitale, Silvia, Duro, Giovanni. 2020. Pompe disease: pathogenesis, molecular genetics and diagnosis. In Aging, 12, 15856-15874. doi:10.18632/aging.103794. https://pubmed.ncbi.nlm.nih.gov/32745073/
2. El Haddad, Léa, Lai, Elias, Murthy, Preetish Kadur Lakshminarasimha, Tata, Purushothama Rao, ElMallah, Mai K. 2023. GAA deficiency disrupts distal airway cells in Pompe disease. In American journal of physiology. Lung cellular and molecular physiology, 325, L288-L298. doi:10.1152/ajplung.00032.2023. https://pubmed.ncbi.nlm.nih.gov/37366541/
3. Li, Zhuoyan, Li, Baolei, Wang, Jing, Yu, Yu, Chen, Sun. 2021. GAA deficiency promotes angiogenesis through upregulation of Rac1 induced by autophagy disorder. In Biochimica et biophysica acta. Molecular cell research, 1868, 118969. doi:10.1016/j.bbamcr.2021.118969. https://pubmed.ncbi.nlm.nih.gov/33513417/
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
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