Mouse β-hexosaminidase A,β-Hex A ELISA Kit

產(chǎn)品詳情

說明書下載

Intra-assay Precision (Precision within an assay): CV%<8%

Three samples of known concentration were tested twenty times on one plate to assess.

Inter-assay Precision (Precision between assays): CV%<10%

Three samples of known concentration were tested in twenty assays to assess.

產(chǎn)品描述：

小鼠β氨基己糖苷酶A(β-Hex A)酶聯(lián)免疫試劑盒（CSB-E04818m）為雙抗夾心法ELISA試劑盒，定量檢測血清、血漿、組織勻漿樣本中的HEXA含量。HEXA是一種溶酶體酶，負責分解GM2神經(jīng)節(jié)苷脂，維持細胞內環(huán)境穩(wěn)定。其突變會導致GM2神經(jīng)節(jié)苷脂在細胞內積累，引發(fā)泰薩二氏病等疾病。研究HEXA的機制，旨在開發(fā)治療相關遺傳性神經(jīng)退行性疾病的藥物。試劑盒檢測范圍為3.12 μU/mL-200 μU/mL，適用于研究β-Hex A在代謝性疾病模型小鼠中的表達調控、基因敲除/過表達動物模型的酶活性驗證，以及藥物干預或病理條件下溶酶體功能的評估等科研場景。本品僅用于科研，不用于臨床診斷，產(chǎn)品具體參數(shù)及操作步驟詳見產(chǎn)品說明書。
別名：

Hexa ELISA Kit; Beta-hexosaminidase subunit alpha ELISA Kit; EC 3.2.1.52 ELISA Kit; Beta-N-acetylhexosaminidase subunit alpha ELISA Kit; Hexosaminidase subunit A ELISA Kit; N-acetyl-beta-glucosaminidase subunit alpha ELISA Kit
縮寫：

HEXA
Uniprot No.：

P29416
種屬：

Mus musculus (Mouse)
樣本類型：

serum, plasma, tissue homogenates
檢測范圍：

3.12 μU/mL-200 μU/mL
靈敏度：

0.78 μU/mL
反應時間：

1-5h
樣本體積：

50-100ul
檢測波長：

450 nm
研究領域：

Neuroscience
測定原理：

quantitative
測定方法：

Sandwich

精密度：

線性度：

To assess the linearity of the assay, samples were spiked with high concentrations of mouse β-Hex A in various matrices and diluted with the Sample Diluent to produce samples with values within the dynamic range of the assay.
	Sample	Serum(n=4)
1:10	Average %	92
	Range %	85-100
1:20	Average %	93
	Range %	87-98
1:40	Average %	90
	Range %	86-100
1:80	Average %	90
	Range %	86-98

回收率：

The recovery of mouse β-Hex A spiked to levels throughout the range of the assay in various matrices was evaluated. Samples were diluted prior to assay as directed in the Sample Preparation section.

Sample Type	Average % Recovery	Range
Serum (n=5)	97	89-101
EDTA plasma (n=4)	95	92-98

標準曲線：

These standard curves are provided for demonstration only. A standard curve should be generated for each set of samples assayed.

功能：

Hydrolyzes the non-reducing end N-acetyl-D-hexosamine and/or sulfated N-acetyl-D-hexosamine of glycoconjugates, such as the oligosaccharide moieties from proteins and neutral glycolipids, or from certain mucopolysaccharides. The isozyme S is as active as the isozyme A on the anionic bis-sulfated glycans, the chondroitin-6-sulfate trisaccharide (C6S-3), and the dermatan sulfate pentasaccharide, and the sulfated glycosphingolipid SM2. The isozyme B does not hydrolyze each of these substrates, however hydrolyzes efficiently neutral oligosaccharide. Only the isozyme A is responsible for the degradation of GM2 gangliosides in the presence of GM2A.
基因功能參考文獻：
1. Neu4 is not the only sialidase contributing to the metabolic bypass in Hexa(-/-) mice. PMID: 20862357
亞細胞定位：

Lysosome.
蛋白家族：

Glycosyl hydrolase 20 family
組織特異性：

Ubiquitous. Most abundant in testis, adrenal, epididymis and heart. Low levels seen in the liver.
數(shù)據(jù)庫鏈接：

KEGG: mmu:15211

STRING: 10090.ENSMUSP00000026262

UniGene: Mm.2284