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Invitrogen™ HMBS Polyclonal Antibody
Rabbit Polyclonal Antibody
Brand: Invitrogen™ PA552253
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Beskrivning
Immunogen sequence: QFEIIAMSTT GDKILDTALS KIGEKSLFTK ELEHALEKNE VDLVVHSLKD LPTVLPPGFT IGAICKRENP HDAVVFHPKF VGKTLETLPE KSVVGTSSLR RAAQLQRKFP HLEFRSIRGN LNTRL Highest antigen sequence identity to the following orthologs: Mouse - 94%, Rat - 94%.
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.
Specifikationer
| HMBS | |
| Polyclonal | |
| Unconjugated | |
| Hmbs | |
| alternative name: porphobilinogen deaminase; HEM3; hemC; HMBS; Hydroxymethylbilane synthase; PBG D; PBGD; PBG-D; PORC; Porphobilinogen deaminase; porphyria, acute; Chester type; pre-uroporphyrinogen synthase; similar to Porphobilinogen deaminase (Hydroxymethylbilane synthase) (HMBS) (Pre-uroporphyrinogen synthase) (PBG-D); T25658; Ups; uroporphyrinogen I synthase; uroporphyrinogen I synthetase; URO-S; Uros1 | |
| Rabbit | |
| Antigen affinity chromatography | |
| RUO | |
| 15288, 25709, 3145 | |
| Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles. | |
| Liquid |
| Immunohistochemistry (Paraffin), Western Blot | |
| 0.3 mg/mL | |
| PBS with 40% glycerol and 0.02% sodium azide; pH 7.2 | |
| P08397, P19356, P22907 | |
| Hmbs | |
| Recombinant protein corresponding to Human HMBS. Recombinant protein control fragment (Product #RP-89538). | |
| 100 μL | |
| Primary | |
| Human, Mouse, Rat | |
| Antibody | |
| IgG |
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