HMBS Antibody (YA9671)
(Synonyms: PBG-D, PBGD, PORC, UPS)HMBS Antibody (YA9671) is a Mouse-derived and non-conjugated IgG1 Monoclonal antibody, targeting to HMBS.
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Host:
Mouse
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Isotype:
IgG
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Application:
IHC-P
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Reactivity :
Human, Mouse, Rat
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Formulation:
Supplied in TBS (pH7.4), 0.05% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.
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Conjugation:
Non-conjugated
To place orders, for customer services and technical support, please contact: MedChemExpress USA
Tel: 609-228-6898 E-mail: [email protected] [email protected]
Applications
| Application |
IHC-P
IHC-P: Immunohistochemistry-Paraffin
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|---|---|
| Dilution Ratio | 1:200 |
Product Details
HMBS Antibody (YA9671) is a Mouse-derived and non-conjugated IgG1 Monoclonal antibody, targeting to HMBS.
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Host Mouse
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Clonality Monoclonal
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Species ReactivityHuman, Mouse, Rat
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Calculated Molecular Weight Predicted band size: 39.3kDa;
Entrez Gene: 3145
Recombinant protein of human HMBS (Full length) produced in HEK293T cell.
Endogenous
affinity purified.
Non-conjugated
Unmodified
IgG
Product Properties
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Appearance
Solution
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Formulation
Supplied in TBS (pH7.4), 0.05% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.
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Storage & Stability
Stored at -20°C for 1 year. Avoid repeated freeze / thaw cycles.
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Shipping
Shipping with blue ice.
Background
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Function
HMBS is an As part of the heme biosynthetic pathway, catalyzes the sequential polymerization of four molecules of porphobilinogen to form hydroxymethylbilane, also known as preuroporphyrinogen. Catalysis begins with the assembly of the dipyrromethane cofactor by the apoenzyme from two molecules of porphobilinogen or from preuroporphyrinogen. The covalently linked cofactor acts as a primer, around which the tetrapyrrole product is assembled. In the last step of catalysis, the product, preuroporphyrinogen, is released, leaving the cofactor bound to the holodeaminase intact[1][2][3][4].
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Subcellular Localization
Cytoplasm, cytosol
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Expression
Tissue_Specificity: Is ubiquitously expressed. -
Isoforms & Post-Translational Modification
HMBS has 4 isoforms, P08397-1: amino acid length is 361, molecular weight is 39330 Da (predicted); P08397-2: amino acid length is 344, molecular weight is 37699 Da (predicted); P08397-3: amino acid length is 321, molecular weight is 34895 Da (predicted); P08397-4: amino acid length is 304, molecular weight is 33264 Da (predicted).
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Subunit
Monomer
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SwissProt ID
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Synonyms
PBG-D, PBGD, PORC, UPS
Documentation
[1]. Cunha L, et al. Human uroporphyrinogen III synthase: NMR-based mapping of the active site. Proteins. 2008 May 1;71(2):855-73. [Content Brief]
[2]. Song G, et al. Structural insight into acute intermittent porphyria. FASEB J. 2009 Feb;23(2):396-404. [Content Brief]
[3]. Ulbrichova D, et al. Correlation between biochemical findings, structural and enzymatic abnormalities in mutated HMBS identified in six Israeli families with acute intermittent porphyria. Blood Cells Mol Dis. 2009 Mar-Apr;42(2):167-73. [Content Brief]
[4]. Bustad HJ, et al. Conformational stability and activity analysis of two hydroxymethylbilane synthase mutants, K132N and V215E, with different phenotypic association with acute intermittent porphyria. Biosci Rep. 2013 Aug 8;33(4):. [Content Brief]
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