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HMBS Recombinant Rabbit Monoclonal Antibody [SC65-07] (ET1610-91)

Price: Regular price $385.00

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Catalog# ET1610-91

Rabbit Monoclonal to HMBS

Tested for

  • WB

Reacts with

  • Human

  • Mouse

  • Rat

Recombinant production enables lot-to-lot consistency and is animal-cruelty-free

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Specifications

Product Type

Recombinant Rabbit monoclonal primary

Product Name

HMBS Recombinant Rabbit Monoclonal Antibody [SC65-07] (ET1610-91)

Immunogen

Recombinant protein within human hmbs aa 1-132 / 299.

Host

Rabbit

Positive Control

Hela cell lysate, 293T cell lysate, K562 cell lysates.

Conjugation

Unconjugated

Clonality

Monoclonal

Clone Number

SC65-07

RRID

AB_3069978

APPLICATION DILUTION

  • WB

  • 1:500

PROPERTIES

Form

Liquid

Storage Condition

Shipped at 4℃. Store at +4℃ short term (1-2 weeks). It is recommended to aliquot into single-use upon delivery. Store at -20℃ long term.

Storage Buffer

1*TBS (pH7.4), 0.05% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.

Concentration

1ug/ul

Purification

Protein A affinity purified.

Molecular Weight

Predicted band size: 39 kDa

Isotype

IgG

TARGET

UNIPROT #

P08397

PROTEIN NAME

HMBS

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;Pre uroporphyrinogen synthase antibody;Pre-uroporphyrinogen synthase antibody;UPS antibody;Uroporphyrinogen I synthase antibody;Uroporphyrinogen I synthetase antibody

SEQUENCE SIMILARITIES

Belongs to the HMBS family.

TISSUE SPECIFICITY

Isoform 1 is ubiquitously expressed. Isoform 2 is found only in erythroid cells.

SUBCELLULAR LOCATION

Cytoplasm.

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.

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