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HMBS, Monoclonal Antibody

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产品名称: HMBS, Monoclonal Antibody
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简单介绍

HMBS, Monoclonal Antibody


HMBS, Monoclonal Antibody  的详细介绍
Product Name

HMBS, Monoclonal Antibody

Full Product Name

Anti-HMBS Rabbit Monoclonal Antibody

Product Synonym Names
Porphobilinogen deaminase; PBG-D; 2.5.1.61; Hydroxymethylbilane synthase; HMBS; Pre-uroporphyrinogen synthase; HMBS; PBGD; UPS
Product Gene Name

anti-HMBS antibody

[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
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OMIM
X04217 mRNA
3D Structure
ModBase 3D Structure for P08397
Clonality
Monoclonal
Host
Rabbit
Species Reactivity
Human, Mouse, Rat
Purity/Purification
Affinity-chromatography
Form/Format
Liquid
Immunogen
A synthesized peptide derived from human HMBS
Subcellular Localization
Cytoplasm.
Tissue Specificity
Isoform 1 is ubiquitously expressed. Isoform 2 is found only in erythroid cells.
Contents
Rabbit IgG in phosphate buffered saline, pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
Preparation and Storage
Store at -20 degree C for one year. After reconstitution, at 4 degree C for one month. It can also be aliquotted and stored frozen at -20 degree C for a longer time. Avoid repeated freezing and thawing.
ISO Certification
Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.
Other Notes
Small volumes of anti-HMBS antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
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Related Product Information for
anti-HMBS antibody
Tetrapolymerization of the monopyrrole PBG into the hydroxymethylbilane pre-uroporphyrinogen in several discrete steps.
Product Categories/Family for anti-HMBS antibody
Signal Transduction; Metabolism; Vitamins/Minerals; Cell Biology; Other Antibodies; Metabolism; Pathways and Processes; Cofactors, vitamins/minerals
Applications Tested/Suitable for anti-HMBS antibody
Western Blot (WB)
Application Notes for anti-HMBS antibody
WB: 1:500-1:2000

Western Blot (WB) of anti-HMBS antibody
Western blot analysis of HMBS expression in HepG2 cell lysate (MBS179678).
Electrophoresis was performed on a 5-20% SDS-PAGE gel at 70V (Stacking gel) / 90V (Resolving gel) for 2-3 hours. The sample well of each lane was loaded with 50ug of sample under reducing conditions.
After Electrophoresis, proteins were transferred to a Nitrocellulose membrane at 150mA for 50-90 minutes. Blocked the membrane with 5% Non-fat Milk/ TBS for 1.5 hour at RT. The membrane was incubated with rabbit anti-HMBS monoclonal antibody overnight at 4 degree C, then washed with TBS-0.1%Tween 3 times with 5 minutes each and probed with a goat anti-rabbit IgG-HRP secondary antibody at a dilution of 1:10000 for 1.5 hour at RT. The signal is developed using an Enhanced Chemiluminescent detection (ECL) kit with Tanon 5200 system. A specific band was detected for HMBS
anti-HMBS antibody Western Blot (WB) (WB) image
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NCBI/Uniprot data below describe general gene information for HMBS. It may not necessarily be applicable to this product.
NCBI GI #
20149500
NCBI GeneID
3145
NCBI Accession #
NP_000181.2 [Other Products]
NCBI GenBank Nucleotide #
NM_000190.3 [Other Products]
UniProt Primary Accession #
P08397 [Other Products]
UniProt Secondary Accession #
P08396; Q16012; A8K2L0; G3V1P4; G5EA58[Other Products]
UniProt Related Accession #
P08397[Other Products]
Molecular Weight
39330 MW
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NCBI Official Full Name
porphobilinogen deaminase isoform 1
NCBI Official Synonym Full Names
hydroxymethylbilane synthase
NCBI Official Symbol
HMBS  [Similar Products]
NCBI Official Synonym Symbols
UPS; PBGD; PORC; PBG-D
  [Similar Products]
NCBI Protein Information
porphobilinogen deaminase
UniProt Protein Name
Porphobilinogen deaminase
UniProt Synonym Protein Names
Hydroxymethylbilane synthase; HMBS; Pre-uroporphyrinogen synthase
UniProt Gene Name
HMBS  [Similar Products]
UniProt Synonym Gene Names
PBGD; UPS; PBG-D; HMBS  [Similar Products]
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NCBI Summary for HMBS
This gene encodes a member of the hydroxymethylbilane synthase superfamily. The encoded protein is the third enzyme of the heme biosynthetic pathway and catalyzes the head to tail condensation of four porphobilinogen molecules into the linear hydroxymethylbilane. Mutations in this gene are associated with the autosomal dominant disease acute intermittent porphyria. Alternatively spliced transcript variants encoding different isoforms have been described. [provided by RefSeq, Jul 2008]
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UniProt Comments for HMBS
Tetrapolymerization of the monopyrrole PBG into the hydroxymethylbilane pre-uroporphyrinogen in several discrete steps.
Research Articles on HMBS
1. A in the HMBS gene confirming the diagnosis of Acute Intermittent Porphyria (AIP)">After a diagnostic odyssey, his urine porphobilinogen was found to be significantly elevated and genetic testing showed a previously unreported consensus splice-site mutation IVS4-1G>A in the HMBS gene confirming the diagnosis of Acute Intermittent Porphyria (AIP)
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Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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