Complement C8 deficient serum human
SIGMA/C1538 - for complement assays
Product Type: Chemical
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| biological source | human |
| form | solution |
| impurities | infectious agent, tested |
| origin | USA origin |
| Quality Level | 200  |
| shipped in | dry ice |
| storage temp. | −70°C |
| technique(s) | activity assay: suitable |
| UniProt accession no. | P07357  |
| Analysis Note: | C8 is depleted by immunoadsorption as judged by a highly sensitive hemolytic assay. |
| Application: | Complement C8 is one of the end terminals of the complement system contained in the membrane attack complex (MAC). A deficiency of C8 may result in an increased susceptibility to Neisseria meningitidis. The most common mutation resulting in a C8 deficiency is a C to T transition in exon 9 of the C8 beta gene. Recent research however, has also discovered that two separate heterogeneous mutations may result in C8 deficiency in patients displaying recurrent meningococcal meningitis. These mutations are a duplication mutation on exon 7 and a mutation on exon 3. |
| Biochem/physiol Actions: | Serum naturally deficient in C8 may actually contain some C8, though in greatly reduced quantity. Terminal complement complex is present at trace levels in such sera. |
| Disclaimer: | RESEARCH USE ONLY. This product is regulated in France when intended to be used for scientific purposes, including for import and export activities (Article L 1211-1 paragraph 2 of the Public Health Code). The purchaser (i.e. enduser) is required to obtain an import authorization from the France Ministry of Research referred in the Article L1245-5-1 II. of Public Health Code. By ordering this product, you are confirming that you have obtained the proper import authorization. |
| Physical form: | Supplied as a solution in PBS, pH 7.3 |
| RIDADR | NONH for all modes of transport |
| WGK Germany | WGK 3 |
| Flash Point(F) | Not applicable |
| Flash Point(C) | Not applicable |
| Storage Temp. | −70°C |
| UNSPSC | 12352202 |