HMBS Recombinant Rabbit Monoclonal Antibody [SC65-07]
Catalog# ET1610-91
HMBS Recombinant Rabbit Monoclonal Antibody [SC65-07]
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WB
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Human
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Mouse
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Rat
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unconjugated
Overview
Product Name
HMBS Recombinant Rabbit Monoclonal Antibody [SC65-07]
Antibody Type
Recombinant Rabbit monoclonal Antibody
Immunogen
Recombinant protein within Human HMBS aa 1-132 / 299.
Species Reactivity
Human (Predicted: Mouse, Rat)
Predicted species support after-sales service
Validated Applications
WB
Target Molecular Weight
Predicted band size: 39 kDa
Positive Control
Hela cell lysate, 293T cell lysate, K562 cell lysates.
Conjugation
unconjugated
Clone Number
SC65-07
RRID
Product Features
Form
Liquid
Concentration
1 mg/mL.(The concentration of this product may be batch-dependent)
Storage Instructions
Shipped at 4℃. Store at +4℃ short term (1-2 weeks). Store at -20℃ long term.
Storage Buffer
1*PBS (pH7.4), 0.1% BSA, 40% Glycerol, 0.2% Proclean 950.
Isotype
IgG
Purification Method
Protein A affinity purified.
Application Dilution
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WB
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1:500
Target
Function
PBGD (porphobilinogen deaminase), also designated hydroxymethylbilane synthase, is a cytoplasmic enzyme found in the heme synthesis pathway. PBGD belongs to the HMBS (hydroxymethylbilane synthase) family. Deficiency of PBGD causes errors in pyrrole metabolism, which in turn leads to an inherited autosomal disorder called acute intermittent porphyria (AIP). AIP is characterized by acute attacks of neurological dysfunctions with hypertension, tachycardia, peripheral neurologic disturbances, abdominal pain and excessive amounts of aminolevulinic acid and porphobilinogen in the urine.
Background References
1. Yang J., et al. 2015. A novel mutation in the porphobilinogen deaminase gene in an extended Chinese family with acute intermittent porphyria. Gene 565:288-290.
2. Gonzaga A.D., et al. 2015. Hydroxymethylbilane synthase gene mutations and polymorphisms in Brazilian families with acute intermittent porphyria. Ann. Hum. Genet. 79:162-172.
Sequence Similarity
Belongs to the HMBS family.
Tissue Specificity
Isoform 1 is ubiquitously expressed. Isoform 2 is found only in erythroid cells.
Subcellular Location
Cytoplasm.
Synonyms
HEM3_HUMAN antibody
HMBS antibody
Hydroxymethylbilane synthase antibody
PBG D antibody
PBG-D antibody
PBGD antibody
PORC antibody
Porphobilinogen deaminase antibody
porphyria, acute
Chester type antibody
ExpandImages
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Western blot analysis of HMBS on different lysates. Proteins were transferred to a PVDF membrane and blocked with 5% BSA in PBS for 1 hour at room temperature. The primary antibody (ET1610-91, 1/500) was used in 5% BSA at room temperature for 2 hours. Goat Anti-Rabbit IgG - HRP Secondary Antibody (HA1001) at 1:200,000 dilution was used for 1 hour at room temperature.
Positive control:
Lane 1: Hela cell lysate
Lane 2: 293T cell lysate -
Western blot analysis of HMBS on K562 cell lysates with Rabbit anti-HMBS antibody (ET1610-91) at 1/500 dilution.
Lysates/proteins at 10 µg/Lane.
Predicted band size: 39 kDa
Observed band size: 41 kDa
Exposure time: 30 seconds;
10% SDS-PAGE gel.
Proteins were transferred to a PVDF membrane and blocked with 5% NFDM/TBST for 1 hour at room temperature. The primary antibody (ET1610-91) at 1/500 dilution was used in 5% NFDM/TBST at room temperature for 2 hours. Goat Anti-Rabbit IgG - HRP Secondary Antibody (HA1001) at 1:200,000 dilution was used for 1 hour at room temperature.
Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"