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Invitrogen
HMBS Polyclonal Antibody
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Product Details
PA5-62230
Applications
Tested Dilution
Publications
Product Specifications
Species Reactivity
Human
Host/Isotype
Rabbit
/ IgG
Class
Polyclonal
Type
Antibody
Immunogen
Recombinant protein corresponding to Human HMBS. Recombinant protein control fragment (Product #RP-89537).
Conjugate
Form
Liquid
Concentration
0.4 mg/mL
Purification
Antigen affinity chromatography
Storage buffer
PBS, pH 7.2, with 40% glycerol
Contains
0.02% sodium azide
Storage conditions
Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
Shipping conditions
Ambient (domestic); Wet ice (international)
RRID
Product Specific Information
Immunogen sequence: EFSAIILATA GLQRMGWHNR VGQILHPEEC MYAVGQGALG VEVRAKDQDI LDLVGVLHDP ETLLRCIAER AFLRHLEGGC SVPVAVHTAM KDGQLYLTGG VWSLDGSDSI QETMQATIHV PAQHEDGPED DPQLV
Highest antigen sequence identity to the following orthologs: Mouse - 93%, Rat - 94%.
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: 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: HMBS; PBG-D; PBGD; PORC; UPS
UniProt ID: (Human) P08397
Entrez Gene ID: (Human) 3145
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
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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