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Product Details
MA5-32253
Product Specifications
Species Reactivity
Human,
Mouse,
Rat
Host/Isotype
Rabbit
/ IgG
Expression System
HEK293 cells
Class
Recombinant Monoclonal
Type
Antibody
Clone
SC65-07
Immunogen
Recombinant protein within Human HMBS aa 1-132
Conjugate
Form
Liquid
Concentration
1 mg/mL
Purification
Protein A
Storage buffer
TBS, pH 7.4, with 40% Glycerol, 0.05% BSA
Contains
0.05% sodium azide
Storage conditions
Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
Shipping conditions
Wet ice
RRID
AB_2809539
Product Specific Information
Recombinant rabbit monoclonal antibodies are produced using in vitro expression systems. The expression systems are developed by cloning in the specific antibody DNA sequences from immunoreactive rabbits. Then, individual clones are screened to select the best candidates for production. The advantages of using recombinant rabbit monoclonal antibodies include: better specificity and sensitivity, lot-to-lot consistency, animal origin-free formulations, and broader immunoreactivity to diverse targets due to larger rabbit immune repertoire.
Target Information
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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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Bioinformatics
Protein Aliases: alternative name: porphobilinogen deaminase; HEM3; HMBS; Hydroxymethylbilane synthase; PBG D; PBG-D; Porphobilinogen deaminase; porphyria, acute; Chester type; Pre-uroporphyrinogen synthase; uroporphyrinogen I synthase; uroporphyrinogen I synthetase
Gene Aliases: hemC; HMBS; PBG-D; PBGD; PORC; T25658; UPS; URO-S; Uros1
UniProt ID: (Human) P08397, (Mouse) P22907
Entrez Gene ID: (Human) 3145, (Mouse) 15288, (Rat) 25709
response to hypoxia
protoporphyrinogen IX biosynthetic process
heme biosynthetic process
response to carbohydrate
response to zinc ion
peptidyl-pyrromethane cofactor linkage
organ regeneration
response to cobalt ion
response to estradiol
response to vitamin
response to drug
response to amino acid
astrocyte differentiation
response to methylmercury
cellular response to antibiotic
cellular response to arsenic-containing substance
cellular response to lead ion
cellular response to cytokine stimulus
cellular response to amine stimulus
cellular response to dexamethasone stimulus
porphyrin-containing compound biosynthetic process
tetrapyrrole biosynthetic process
response to hormone
response to metal ion
response to lead ion
response to organic cyclic compound
response to nutrient levels
Performance Guarantee
If an Invitrogen™ antibody doesn't perform as described on our website or datasheet,we'll replace the product at no cost to you, or provide you with a credit for a future purchase.*
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