Analytical Data
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基因名
HMBS/Porphobilinogen deaminase
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简介
HMBS (porphobilinogen deaminase) is key to heme biosynthesis, catalyzing the sequential polymerization of four porphobilinogen molecules to form hydroxymethylbiphenyl. The process begins with dipyrromethane cofactor assembly derived from porphobilinogen or preuroporphyrinogen and promoted by apoenzymes. HMBS/Porphobilinogen deaminase Protein, Human (HEK293, His) is the recombinant human-derived HMBS/Porphobilinogen deaminase protein, expressed by HEK293 , with C-6*His labeled tag.
- Application
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别名
rHuPorphobilinogen deaminase/HMBS, His; Porphobilinogen Deaminase; PBG-D; Hydroxymethylbilane Synthase; HMBS; Pre-Uroporphyrinogen Synthase; HMBS; PBGD; UPS
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种属
Human
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表达系统
HEK293
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标签
C-6*His
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纯度
Greater than 90% as determined by SDS-PAGE.
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蛋白编号
P08397-1
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表达区间
S2-H361
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氨基酸序列
SGNGNAAATAEENSPKMRVIRVGTRKSQLARIQTDSVVATLKASYPGLQFEIIAMSTTGDKILDTALSKIGEKSLFTKELEHALEKNEVDLVVHSLKDLPTVLPPGFTIGAICKRENPHDAVVFHPKFVGKTLETLPEKSVVGTSSLRRAAQLQRKFPHLEFRSIRGNLNTRLRKLDEQQEFSAIILATAGLQRMGWHNRVGQILHPEECMYAVGQGALGVEVRAKDQDILDLVGVLHDPETLLRCIAERAFLRHLEGGCSVPVAVHTAMKDGQLYLTGGVWSLDGSDSIQETMQATIHVPAQHEDGPEDDPQLVGITARNIPRGPQLAAQNLGISLANLLLSKGAKNILDVARQLNDAH
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蛋白长度
Full Length of Isoform-1 Mature Protein
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分子量
47.0 kDa
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内毒素
< 1.0 EU per μg protein as determined by the LAL method.
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性状
Freeze-dried powder
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缓冲液
PBS, pH7.4, containing 0.01% SKL, 1mM DTT, 5% Trehalose and Proclin300.
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复溶方法
Reconstitute in ddH2O to a concentration of 0.1-0.5 mg/mL. Do not vortex.
- 个性化定制
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稳定性测试
The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37℃ for 48h, and no obvious degradation and precipitation were observed. The loss rate isless than 8% within the expiration date under appropriate storage condition.
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保存条件 & 期限
Samples are stable for up to twelve months from date of receipt at -20℃ to -80℃. Store it under sterile conditions at -20℃ to -80℃. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles.
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运输条件
In general, recombinant proteins are supplied as lyophilized powder and shipped at ambient temperature. For bulk packages, the proteins are provided as frozen liquid and shipped with blue ice, unless otherwise requested by the customer.
Quality inspection process
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Protein Description
HMBS (Hydroxymethylbilane Synthase) is an enzyme that plays a crucial role in the heme biosynthesis pathway, catalyzing the conversion of porphobilinogen into hydroxymethylbilane. Deficiencies in HMBS, often due to mutations in the HMBS gene, can lead to acute intermittent porphyria (AIP), a genetic disorder characterized by the accumulation of porphyrins and their precursors, resulting in severe abdominal pain, neurological symptoms, and various complications. Understanding the structure and function of recombinant HMBS/Porphobilinogen deaminase is pivotal for elucidating the mechanisms behind AIP and other porphyrias. Recombinant protein studies facilitate the exploration of enzyme kinetics, substrate specificity, and the impact of specific mutations on enzyme activity. Furthermore, producing active recombinant HMBS provides a valuable tool for drug discovery and the development of therapeutic strategies aimed at managing porphyria episodes and mitigating symptoms. Advances in recombinant DNA technology enable the generation of high-yield enzyme systems, allowing for detailed biochemical assays and the potential identification of small molecules that could be used to treat or prevent enzyme deficiencies. Overall, research into recombinant HMBS not only aids in comprehending fundamental biological processes but also paves the way for innovative treatments that address the clinical challenges posed by porphyrias.
