SLC25A15 Antibody

說明書

中文名稱：

SLC25A15兔多克隆抗體
貨號(hào)：

CSB-PA897578ESR1HU
規(guī)格：

￥440
圖片：
Western blot
All lanes: SLC25A15 antibody at 6μg/ml + Mouse liver tissue
Secondary
Goat polyclonal to rabbit IgG at 1/10000 dilution
Predicted band size: 33 kDa
Observed band size: 33 kDa

Immunohistochemistry of paraffin-embedded human placenta tissue using CSB-PA897578ESR1HU at dilution of 1:100
其他：

產(chǎn)品詳情
產(chǎn)品評(píng)價(jià)
相關(guān)產(chǎn)品
靶點(diǎn)詳情

產(chǎn)品詳情

產(chǎn)品名稱：

Rabbit anti-Homo sapiens (Human) SLC25A15 Polyclonal antibody
Uniprot No.：

Q9Y619
基因名：

SLC25A15
別名：

HHH antibody; Mitochondrial ornithine transporter 1 antibody; ORC1 antibody; Ornithine transporter 1 antibody; Ornithine transporter, mitochondrial antibody; ORNT1 antibody; ORNT1_HUMAN antibody; SLC25A15 antibody; Solute carrier family 25 (Mitochondrial carrier, ornithine transporter) member 15 antibody; Solute carrier family 25 member 15 antibody
宿主：

Rabbit
反應(yīng)種屬：

Human, Mouse
免疫原：

Recombinant Human Mitochondrial ornithine transporter 1 protein (1-301AA)
免疫原種屬：

Homo sapiens (Human)
標(biāo)記方式：

Non-conjugated
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

Antigen Affinity Purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
產(chǎn)品提供形式：

Liquid
應(yīng)用范圍：

ELISA, WB, IHC

推薦稀釋比：

Application	Recommended Dilution
WB	1:200-1:1000
IHC	1:20-1:200

Protocols：

ELISA Protocol
Western Blotting (WB) Protocol
Immunohistochemistry (IHC) Protocol
儲(chǔ)存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產(chǎn)品評(píng)價(jià)

靶點(diǎn)詳情

功能：

Ornithine-citrulline antiporter. Connects the cytosolic and the intramitochondrial reactions of the urea cycle by exchanging cytosolic ornithine with matrix citrulline. The stoichiometry is close to 1:1.
基因功能參考文獻(xiàn)：
1. characterized mutations of the proposed substrate binding site in ORC1 and ORC2; demonstrated that the residue at position 179 in the 2 soforms is largely responsible for the difference in their substrate specificity;concluded that Arg-179 is a key residue in the opening of the carrier to the matrix side PMID: 22262851
2. Mutation analysis revealed two novel mutations in the ORNT1 gene. PMID: 22465082
3. Useful insights for in-depth understanding of the molecular mechanism of the HHH syndrome and developing effective drugs against the disease. PMID: 22292090
4. novel mutations in patients with hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome PMID: 11668643
5. expression, reconstitution, functional characterization, and tissue distribution of two human isoforms PMID: 12807890
6. Hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome with evidence of mitochondrial dysfunction due to a novel SLC25A15 (ORNT1) gene mutation in a Palestinian family. PMID: 14759633
7. The DeltaF 188 mutant was not incorporated into the membrane to the same extent as wild type, but retained significant residual activity and lost stereospecificity. PMID: 16256388
8. A novel R275X mutation of the SLC25A15 gene in a Japanese patient with the HHH syndrome PMID: 16376511
9. The three patients were homozygous for a novel mutation in ORNT1 with a Gly220Arg change. We suggest including HHH syndrome in the differential diagnosis of patients found to have stroke-like lesions on brain MRI. PMID: 17825324
10. Clinical presentations and outcomes varied significantly in HHH syndrome patients homozygous for delF188 mutations in SLC25A15. PMID: 18978333
11. 16 additional Hyperornithinemia-hyperammonemia-homocitrullinuria cases were collected and the spectrum of SLC25A15/ORC1 mutations, was expanded. PMID: 19242930
顯示更多

收起更多
相關(guān)疾病：

Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome (HHH syndrome)
亞細(xì)胞定位：

Mitochondrion inner membrane; Multi-pass membrane protein.
蛋白家族：

Mitochondrial carrier (TC 2.A.29) family
組織特異性：

Highly expressed in liver, pancreas, testis, lung and small intestine. Lower levels are detected in spleen, kidney, brain and heart.
數(shù)據(jù)庫鏈接：

HGNC: 10985

OMIM: 238970

KEGG: hsa:10166

STRING: 9606.ENSP00000342267

UniGene: Hs.646645

Most popular with customers

YWHAG Recombinant Monoclonal Antibody

Applications: ELISA, WB, IHC

Species Reactivity: Human
YWHAB Recombinant Monoclonal Antibody

Applications: ELISA, WB, IHC, IF, FC

Species Reactivity: Human, Mouse, Rat
Phospho-YAP1 (S127) Recombinant Monoclonal Antibody

Applications: ELISA, WB, IHC

Species Reactivity: Human
XRCC5 Recombinant Monoclonal Antibody

Applications: ELISA, WB, IHC, IF

Species Reactivity: Human
XPNPEP1 Recombinant Monoclonal Antibody

Applications: ELISA, IHC, FC

Species Reactivity: Human
XDH Recombinant Monoclonal Antibody

Applications: ELISA, WB, IHC

Species Reactivity: Human
WT1 Recombinant Monoclonal Antibody

Applications: ELISA, WB, IHC

Species Reactivity: Human
WFDC2 Recombinant Monoclonal Antibody

Applications: ELISA, WB, FC

Species Reactivity: Human