구독하기
연구 모델
서비스
전임상 효능 평가
Resource
huSLC6A19/Pah-R243Q Mouse
제품 견적 요청
카탈로그에서 제품을 선택하여 요청을 제출해 주세요. Cyagen 팀이 상세 정보를 제공해 드립니다.
huSLC6A19/Pah-R243Q Mouse
제품명
huSLC6A19/Pah-R243Q Mouse
제품 ID
C002064
품종 계통
C57BL/6NCya-Slc6a19tm1(hSLC6A19)Pahem2(R243Q)/Cya
Backgroud
C57BL/6NCya
상태
이 마우스 계통을 논문에서 사용할 경우, “huSLC6A19/Pah-R243Q Mouse (카탈로그 번호 C002064)은 Cyagen에서 구입하였습니다.”라고 명시해 주시기 바랍니다.
HUGO-GT Humanized Models
Metabolic Target Humanized Mouse Models
Disease Animal Models
구매 가능한 제품 종류
연령
Genotype
성별
수량
표준 제공 조건은 최소 3마리의 이형접합(heterozygous) 보균자를 보장합니다. 동형접합(homozygous) 보균자 및/또는 특정 성별에 대한 브리딩 서비스도 제공됩니다.
가격 문의
HUGO-GT Humanized Models
Metabolic Target Humanized Mouse Models
Disease Animal Models
기본 정보
검증 데이터
관련 자료
기본 정보
유전자 별칭
HND, B0AT1
염색체
Chr 10, Chr 5
MGI ID
Datasheet
품종 계통 설명
The huSLC6A19/Pah-R243Q mouse was generated by replacing the endogenous mouse Slc6a19 gene sequence (from the start codon to the 3'UTR) with the human SLC6A19 gene sequence (from the start codon to the 3'UTR) in the Pah-R243Q mice (catalog No.: C001738). This model is suitable for research on phenylketonuria (PKU), amino acid metabolism disorders, and the screening and preclinical evaluation of SLC6A19 inhibitors.
변형 전략

Figure 1. Gene editing strategy of huSLC6A19 mice. The sequences from the start codon to the 3'UTR of the endogenous mouse Slc6a19 gene were replaced with the sequences from the start codon to the 3'UTR of the human SLC6A19 gene. The expression of mouse Slc6a19os may be affected by this KI region.

Figure 2. Gene editing strategy of Pah-R243Q mice. The p.R243Q (CGT to CAA) point mutation was introduced into exon 7 of the mouse Pah gene.
응용 분야
Screening, development, and preclinical evaluation of SLC6A19 and Pah-R243Q targeted drugs;
Research on phenylketonuria (PKU);
Research on amino acid metabolism disorders;
Screening and preclinical evaluation of SLC6A19 inhibitors.
검증 데이터
1. Sequencing
Sequencing results confirmed the presence of the Pah point mutation (CGT→CAA) in the kidneys and duodenum tissues of homozygous huSLC6A19/Pah-R243Q mice.

Figure 3. Sequencing results of kidney and duodenum tissues from homozygous huSLC6A19/Pah-R243Q and wild-type (WT) mice.
2. Gene Expression
Human SLC6A19 and mouse Slc6a19 transcripts in the kidneys and duodena of double-homozygous huSLC6A19/Pah-R243Q and wild-type (WT) mice were quantified using RT-qPCR with specific primers. Relative expression levels were normalized to mouse β-actin and are presented as mean±SD. RT-qPCR analysis revealed human SLC6A19 transcripts, but no mouse Slc6a19 transcripts, in the kidneys and duodena of huSLC6A19/Pah-R243Q mice. Conversely, WT mice exhibited mouse Slc6a19 transcripts, with no detectable human SLC6A19 transcripts in these tissues.

Figure 4. RT-qPCR analysis of human SLC6A19 and mouse Slc6a19 transcript levels in the kidneys and duodena of huSLC6A19/Pah-R243Q and wild-type (WT) mice (6–7 weeks old, male, homozygous, n=3).
3. Immunohistochemistry (IHC)
IHC staining revealed the expression of human SLC6A19 protein in the kidney tissues of homozygous huSLC6A19/Pah-R243Q mice (red arrows indicate positive signals on the luminal surface of renal tubules), whereas no human SLC6A19 protein was detected in the kidneys of wild-type (WT) mice.

Figure 5. Immunohistochemistry (IHC) results of kidney tissues from huSLC6A19/Pah-R243Q and wild-type (WT) mice (5–7 weeks old, male/female, homozygous, n=2).
문의하기
맞춤형 동물 모델 관련 상담을 위해 Cyagen 전문가와 연락해 보세요. 아래 양식을 작성하여 상담을 시작하거나 견적을 요청하시기 바랍니다.
Cyagen은 고객님의 개인정보를 소중히 여깁니다. 최신 제품, 서비스 및 인사이트를 안내드리고자 합니다. 고객님의 수신 설정은 다음과 같습니다:
해당 커뮤니케이션은 언제든지 수신 거부하실 수 있습니다. 수신 거부 방법 및 데이터 보호에 대한 자세한 내용은 개인정보처리방침을 참고해 주시기 바랍니다.
아래 버튼을 클릭함으로써, 요청하신 콘텐츠 제공을 위해 본 양식을 통해 제출된 개인정보를 Cyagen이 저장 및 처리하는 데 동의하게 됩니다.
