Human N-sulphoglucosamine sulphohydrolase(SGSH) ELISA kit

Human N-sulphoglucosamine sulphohydrolase(SGSH) ELISA kit

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中文名稱：

人磺氨基葡糖硫酸胺酶(SGSH)酶聯(lián)免疫試劑盒
貨號：

CSB-EL021200HU
規(guī)格：

96T/48T
價格：

￥3600/￥2500
其他：

CUSABIO Wins 2024 Bioz Rapid Star? Award in the ELISA category

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產(chǎn)品描述：

人磺氨基葡糖硫酸胺酶(SGSH)酶聯(lián)免疫試劑盒（CSB-EL021200HU）為雙抗夾心法ELISA試劑盒，定量檢測血清、血漿、組織勻漿、細胞裂解物樣本中的SGSH含量。SGSH即N - 磺基葡糖胺磺基水解酶，它參與硫酸乙酰肝素的降解。若SGSH功能缺陷，會使硫酸乙酰肝素在體內(nèi)蓄積，引發(fā)黏多糖貯積癥ⅢA型。目前對其研究多聚焦于酶替代療法等改善病情的機制，以緩解患者癥狀。試劑盒檢測范圍為31.25 pg/mL-2000 pg/mL，適用于科研領(lǐng)域中對SGSH表達調(diào)控機制的研究，例如在疾病模型構(gòu)建、基因編輯細胞系驗證或溶酶體功能相關(guān)分子通路探索中定量分析SGSH的動態(tài)變化，為神經(jīng)退行性疾病、溶酶體貯積癥等基礎(chǔ)研究提供靈敏可靠的工具。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：

Heparan sulfate sulfatase ELISA Kit; Heparan sulphate sulphatase ELISA Kit; HSS ELISA Kit; MPS 3A ELISA Kit; MPS3 A ELISA Kit; MPS3A ELISA Kit; Mucopolysaccharidosis type IIIA ELISA Kit; N sulfoglucosamine sulfohydrolase (sulfamidase) ELISA Kit; N sulfoglucosamine sulfohydrolase ELISA Kit; N-sulphoglucosamine sulphohydrolase ELISA Kit; SFMD ELISA Kit; SGSH ELISA Kit; SPHM_HUMAN ELISA Kit; Sulfoglucosamine sulfamidase ELISA Kit; Sulphamidase ELISA Kit; Sulphoglucosamine sulphamidase ELISA Kit
縮寫：

SGSH
Uniprot No.：

P51688
種屬：

Homo sapiens (Human)
樣本類型：

serum, plasma, tissue homogenates, cell lysates
檢測范圍：

31.25 pg/mL-2000 pg/mL
靈敏度：

7.81 pg/mL
反應(yīng)時間：

1-5h
樣本體積：

50-100ul
檢測波長：

450 nm
研究領(lǐng)域：

Signal Transduction
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 human SGSH 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)
1:1	Average %	92
	Range %	87-97
1:2	Average %	97
	Range %	93-102
1:4	Average %	84
	Range %	81-88
1:8	Average %	89
	Range %	83-94

回收率：

The recovery of human SGSH 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)	101	97-106
EDTA plasma (n=4)	92	89-95

標準曲線：

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

pg/ml	OD1	OD2	Average	Corrected
2000	2.256	2.136	2.196	2.069
1000	1.916	1.788	1.852	1.725
500	1.253	1.355	1.304	1.177
250	0.813	0.809	0.811	0.684
125	0.467	0.505	0.486	0.359
62.5	0.326	0.350	0.338	0.211
31.25	0.222	0.238	0.230	0.103
0	0.124	0.130	0.127

數(shù)據(jù)處理：

ELISA操作視頻及標曲繪制軟件下載
貨期：

3-5 working days

功能：

Catalyzes a step in lysosomal heparan sulfate degradation.
基因功能參考文獻：
1. CSF enzyme activity levels for either SGSH (in MPS IIIA subjects) or NAGLU (in MPS IIIB) significantly differed from normal controls. Several other behavioral or functional measures were found to be uninformative in this population, including timed functional motor tests. PMID: 27590925
2. We have identified ocular features of a patient with Sanfilippo syndrome type IIIA harboring a novel SGHS mutation that were not previously known to occur in this disease - namely, a progressive retinopathy with distinctive features, cystic macular changes responsive to carbonic anhydrase inhibitors, and complex electroretinographic abnormalities consistent with postreceptoral dysfunction. PMID: 26331342
3. results demonstrate that a single systemic scAAVrh74-hSGSH delivery mediated efficient restoration of SGSH activity and resulted in a near complete correction of MPS IIIA molecular pathology PMID: 25592334
4. The crystal structure of glycosylated sulfamidase provides insight into the diverse effects of pathogenic mutations on sulfamidase function in mucopolysaccharidosis type IIIA. PMID: 24816101
5. Pre-symptomatic treatment of progressive neurodegenerative disease (mucopolysaccharidosis type IIIA) via intra-cerebrospinal fluid injection of recombinant human SGSH mediates highly significant reductions in neuropathology in a canine model. PMID: 21550404
6. Processing and secretion of p.Ser298Pro sulfamidase suggests that small amounts of the newly synthesized enzyme are transported to lysosomes PMID: 21671382
7. Molecular genetics of mucopolysaccharidosis type IIIA and IIIB: Diagnostic, clinical, and biological implications PMID: 11668611
8. Sanfilippo syndrome (subtypes A and B) in Turkey: identification of novel mutations in SGSH and NAGLU PMID: 11793481
9. expression studies of four novel mutations PMID: 15146460
10. analysis of a nonsense mutation (Y40X) and two de novo missense mutations (E300V; Q307P) in heparan N-sulphatase in a mucopolysaccharidosis IIIA patient [case report] PMID: 15902564
11. By assessing the degree of developmental regression over time a group of 7 pts with a slowly progressive course of MPSIIIA were identified. In these 7 pts and in 3 other mildly affected pts missense mutation c.892T>C (p.Ser298Pro) was found on 1 allele PMID: 18407553
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相關(guān)疾病：

Mucopolysaccharidosis 3A (MPS3A)
亞細胞定位：

Lysosome.
蛋白家族：

Sulfatase family
數(shù)據(jù)庫鏈接：

HGNC: 10818

OMIM: 252900

KEGG: hsa:6448

STRING: 9606.ENSP00000314606

UniGene: Hs.31074

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.