ALAS1 Recombinant Monoclonal Antibody

說明書

中文名稱：

ALAS1重組抗體
貨號：

CSB-RA266893A0HU
規格：

￥1320
圖片：
Western Blot
Positive WB detected in: Hela whole cell lysate, HepG2 whole cell lysate, Raji whole cell lysate, PC-3 whole cell lysate, A549 whole cell lysate, MCF-7 whole cell lysate
All lanes: ALAS1 antibody at 1:1500
Secondary
Goat polyclonal to rabbit IgG at 1/50000 dilution
Predicted band size: 71, 13 kDa
Observed band size: 71 kDa
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

產品描述：

CSB-RA266893A0HU ALAS1重組單克隆抗體是針對δ-氨基乙酰丙酸合酶1（ALAS1）的高特異性科研工具，該靶標是血紅素生物合成的限速酶，在細胞能量代謝和氧轉運過程中發揮關鍵作用。本抗體通過重組技術制備，具有優異的批次一致性和低交叉反應性，經ELISA與Western Blot（WB）嚴格驗證，在WB實驗中推薦使用1:500至1:5000的寬范圍稀釋度，可清晰檢測內源性ALAS1蛋白表達。其應用場景涵蓋血紅素合成通路機制研究、鐵代謝相關疾病模型分析，以及藥物干預對線粒體功能影響的分子水平評估，適用于肝細胞、紅細胞前體細胞等樣本的蛋白質表達研究。該產品為科研人員探索ALAS1在遺傳性卟啉癥、貧血等病理過程中的作用提供了可靠工具，實驗結果具備高重復性與靈敏度，滿足基礎研究中對靶標蛋白精準檢測的需求。
Uniprot No.：

P13196
基因名：

ALAS1
別名：

5-aminolevulinate synthase, nonspecific, mitochondrial (ALAS-H) (EC 2.3.1.37) (5-aminolevulinic acid synthase 1) (Delta-ALA synthase 1) (Delta-aminolevulinate synthase 1), ALAS1, ALAS3 ALASH
反應種屬：

Human
免疫原：

A synthesized peptide derived from human Alas1
免疫原種屬：

Homo sapiens (Human)
標記方式：

Non-conjugated
克隆類型：

Monoclonal
抗體亞型：

Rabbit IgG
純化方式：

Affinity-chromatography
克隆號：

2A9
濃度：

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

Rabbit IgG in 10mM phosphate buffered saline , pH 7.4, 150mM sodium chloride, 0.05% BSA, 0.02% sodium azide and 50% glycerol.
產品提供形式：

Liquid
應用范圍：

ELISA, WB

推薦稀釋比：

Application	Recommended Dilution
WB	1:500-1:5000

Protocols：

ELISA Protocol
Western Blotting (WB) Protocol
儲存條件：

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.

產品評價

靶點詳情

基因功能參考文獻：
1. The mutation in CLPX inactivates its ATPase activity, resulting in coassembly of mutant and WT protomers to form an enzyme with reduced activity. The presence of low-activity CLPX increases the posttranslational stability of ALAS, causing increased ALAS protein and ALA levels, leading to abnormal accumulation of PPIX. PMID: 28874591
2. the heme-binding site in the N-terminal region of the mature ALAS1 protein is also necessary for the heme-dependent oxidation of ALAS1. PMID: 27496948
3. ALAS1 mRNA and activity were elevated approximately ~3- and 5-fold, and HMB synthase activity was approximately half-normal (~42%) PMID: 26062020
4. The -853T variant functions as an enhancer in the presence of estrogen and speculates that the -1253A variant reduces transcription activity. PMID: 19656447
5. These results indicate that ALAS1 is a novel NR5A-target gene and participates in steroid hormone production. PMID: 23024262
6. Lon peptidase 1 (LONP1)-dependent breakdown of mitochondrial 5-aminolevulinic acid synthase protein by heme in human liver cells. PMID: 21659532
7. REVIEW:active site and mechanistic analysis, protein folding, structure and function. PMID: 11929042
8. REVIEW: mechanisms involving ALAS deficiency, point mutations, post translational processing, and complex formation with succinyl CoA synthetase subunit B in the pathogenesis of hereditary sideroblastic anemia. PMID: 11929048
9. ALAS expression is regulated by AP-1 complex through sequestration of cAMP-response element protein (CRE)-binding protein (CBP) coactivator in human cells PMID: 12433930
10. ALAS gene expression is regulated by Hepatic nuclear factor 3 and nuclear factor 1 PMID: 15123725
11. First described frameshift ALAS2 mutation, CD506-507 (-C). PMID: 15477213
12. in the liver of Acute liver failure patients, there may be an increase in free heme concentration which down-regulating ALAS1 gene expression PMID: 15547665
13. Alternative splicing of human ALAS1 generates two mRNAs with different 5'-UTRs: a major one, where exon 1B is omitted, and a minor form containing exon 1B. PMID: 15710391
14. 5-aminolevulinate synthase gene repression by the potent tumor promoter, TPA, involves multiple signal transduction pathways PMID: 15797241
15. Expression of candidate genes HPRT1 and ALAS1 in malignant and non-malignant prostate tissue samples after microdissection. PMID: 17628775
16. Differential regulation of human ALAS1 mRNA and protein levels by heme and cobalt protoporphyrin. PMID: 18719978
17. In this study, we show significant reductions of the rate-limiting enzymes involved in heme biosynthesis, ALAS1 in the postmortem cortex of Alzheimer's disease subjects, providing additional evidence of abnormal heme homeostasis in Alzheimer's disease. PMID: 19477221
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亞細胞定位：

Mitochondrion matrix.
蛋白家族：

Class-II pyridoxal-phosphate-dependent aminotransferase family
數據庫鏈接：

HGNC: 396

OMIM: 125290

KEGG: hsa:211

STRING: 9606.ENSP00000309259

UniGene: Hs.476308

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