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AASS Polyclonal antibody, PBS Only

AASS Polyclonal Antibody for WB, IHC, IP, Indirect ELISA
Cat No. 29354-1-PBS

产品说明书

宿主/亚型

Rabbit / IgG

种属反应性

human, mouse, rat

应用

WB, IHC, IP, Indirect ELISA

LKR, LKR/SDH, LKRSDH, LOR, LORSDH

缓冲液配方:  PBS Only
偶联物:  Unconjugated
规格价格库存


产品信息

29354-1-PBS targets AASS in WB, IHC, IP, Indirect ELISA applications and shows reactivity with human, mouse, rat samples.

经测试应用 WB, IHC, IP, Indirect ELISA Application Description
经测试反应性 human, mouse, rat
免疫原

CatNo: Ag29520

Product name: Recombinant human AASS protein

Source: e coli.-derived, PGEX-4T

Tag: GST

Domain: 33-146 aa of NM_005763

Sequence: VNAWERRAPLAPKHIKGITNLGYKVLIQPSNRRAIHDKDYVKAGGILQEDISEACLILGVKRPPEEKLMSRKTYAFFSHTIKAQEANMGLLDEILKQEIRLIDYEKMVDHRGVR

种属同源性预测
宿主/亚型 Rabbit / IgG
抗体类别 Polyclonal
产品类型 Antibody
全称 aminoadipate-semialdehyde synthase
别名 LKR, LKR/SDH, LKRSDH, LOR, LORSDH
计算分子量 102 kDa
观测分子量 100-110 kDa
GenBank蛋白编号NM_005763
基因名称 AASS
Gene ID (NCBI) 10157
RRIDAB_3742311
偶联类型 Unconjugated
形式Liquid
纯化方式Antigen affinity purification
UNIPROT IDQ9UDR5
储存缓冲液 PBS only, pH 7.3.
储存条件Store at -80°C.
The product is shipped with ice packs. Upon receipt, store it immediately at -80°C

背景介绍

Aminoadipate-semialdehyde synthase (AASS) is a bifunctional enzyme catalyzing the first two steps of lysine degradation via the saccharopine pathway. It possesses both lysine-ketoglutarate reductase and saccharopine dehydrogenase activities. Mutations in the AASS gene cause familial hyperlysinemia, a rare autosomal recessive disorder characterized by elevated plasma lysine levels, though clinical manifestations are typically benign. AASS dysfunction disrupts mitochondrial energy metabolism and amino acid homeostasis. Beyond its metabolic role, emerging evidence suggests AASS may influence cellular redox status and autophagy regulation, positioning it as a potential therapeutic target for metabolic disorders and lysine-related pathologies (PMID: 41158987; 10775527).

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